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'''For patient information click [[Leukemia (patient information)|here]]'''
__NOTOC__
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{{DiseaseDisorder infobox |
  Name          = Leukemia |
  ICD10          = {{ICD10|C|91||c|81}}-{{ICD10|C|95||c|81}} |
  ICD9          = {{ICD9|208.9}} |
  ICDO          = 9800-9940 |
  Image          = acute_leukemia-ALL.jpg |
  Caption        = A Wright's stained bone marrow aspirate smear of patient with precursor B-cell acute lymphoblastic leukemia.|
  OMIM          = |
  MedlinePlus    = |
  eMedicineSubj  = |
  eMedicineTopic = |
  DiseasesDB    = 7431 |
  MeshID        = D007938 |
}}
{{CMG}}
{{Leukemia}}
{{Leukemia}}
{{Editor Join}}


==Overview==
'''For patient information click [[Leukemia (patient information)|here]]'''
'''Leukemia''' or '''leukaemia''' (Greek leukos, “white”; haima, “blood”) is a [[cancer]] of the [[blood]] or [[bone marrow]] and is characterized by an abnormal proliferation (production by multiplication) of blood [[Cell (biology)|cells]], usually white blood cells ([[leukocytes]]). It is part of the broad group of diseases called [[Hematological malignancy|hematological neoplasms]].


==Symptoms==
{{CMG}}; {{AE}} {{SMP}}, {{USAMA}}, {{SSH}}; {{GRR}} {{Nat}}
Damage to the [[bone marrow]], by way of displacing the normal bone marrow cells with higher numbers of immature white blood cells, results in a lack of blood [[platelet]]s, which are important in the [[Coagulation of human blood|blood clotting]] process. This means people with leukemia may become [[purpura|bruised]], [[hemorrhage|bleed]] excessively, or develop pinprick bleeds ([[petechia]]e).


[[White blood cell]]s, which are involved in fighting [[pathogen]]s, may be suppressed or dysfunctional. This could cause the patient's immune system (white blood cells etc.) to start attacking other body cells.
{{SK}} Leukaemia


Finally, the red blood cell deficiency leads to [[anemia]], which may cause [[dyspnea]]. All symptoms can be attributed to other diseases; for [[diagnosis]], [[blood test]]s and a [[bone marrow examination]] are required.
==Overview==
 
'''Leukemia''' (Greek leukos, “white”; haima, “blood”) can be defined as a group of [[hematopoietic stem cell]] [[malignancies]] due to [[genetic]] abnormalities that may lead to clonal proliferation of these cells. These group of diseases are classified based on the type of [[hematopoietic stem cell]] involved and the duration of the disease. The leukemias are the most common malignancies among children younger than 15 years. Among them, [[ALL|Acute Lymphoblastic Leukemia (ALL)]] is the most common leukemia and accounts for 77% of childhood leukemia. However, [[CLL|Chronic Lymphocytic Leukemia (CLL)]] is the most common form of leukemia in adults, and it accounts for 30% of all leukemias in the United States. The increased rate of proliferation and decreased rate of [[apoptosis]] in this progeny of cells may compromise normal [[bone marrow]] function and ultimately result in marrow failure. Clinical manifestations, diagnosis, laboratory findings, and therapy are different according to the type of malignancy.
Some other related symptoms:
{{div col}}
* [[Fever]], [[chills]], [[night sweat]]s and other [[flu]]-like symptoms
* [[Weakness]] and [[fatigue]]
* Swollen or bleeding gums
* Neurological symptoms ([[headache]]s)
* [[hepatomegaly|Enlarged liver]] and [[splenomegaly|spleen]]
* Frequent [[infection]]
* Bone pain
* [[Joint pain]]
* [[Dizziness]]
* [[Nausea]]
* Swollen [[tonsils]]
* [[Diarrhea]]
* [[Paleness]]
* [[Malaise]]
* Unintentional [[Weight_loss#Unintentional_weight_loss | weight loss]]
{{div col end}}
 
The word leukemia, which means 'white blood,' is derived from the disease's namesake high white blood cell counts that most leukemia patients have before treatment. The high number of white blood cells are apparent when a blood sample is viewed under a microscope.  Frequently, these extra white blood cells are immature or dysfunctional.  The excessive number of cells can also interfere with the normal function of other cells.
 
Some leukemia patients do not have high white blood cell counts visible during a regular blood count.  This less-common condition is called '''aleukemia'''.  The bone marrow still contains cancerous white blood cells which disrupt the normal production of blood cells. However, the leukemic cells are staying in the marrow instead of entering the bloodstream, where they would be visible in a blood test. For an aleukemic patient, the white blood cell counts in the bloodstream can be normal or low.  Aleukemia can occur in any of the four major types of leukemia, and is particularly common in [[hairy cell leukemia]].


==Classification==
==Classification==
Leukemia is clinically and pathologically subdivided into several large groups.  The first division is between its ''[[Acute (medical)|acute]]'' and ''[[chronic (medicine)|chronic]]'' forms:
* ''[[Acute leukemia]]'' is characterized by the rapid increase of immature blood cells. This crowding makes the bone marrow unable to produce healthy blood cells. Acute forms of leukemia can occur in children and young adults. (In fact, it is a more common cause of death for children in the [[United States|US]] than any other type of malignant disease). Immediate treatment is required in acute leukemias due to the rapid progression and accumulation of the malignant cells, which then spill over into the bloodstream and spread to other organs of the body. Central nervous system (CNS) involvement is uncommon, although the disease can occasionally cause cranial nerve palsies.
* ''[[Chronic leukemia]]'' is distinguished by the excessive build up of relatively mature, but still abnormal, blood cells. Typically taking months or years to progress, the cells are produced at a much higher rate than normal cells, resulting in many abnormal white blood cells in the blood. Chronic leukemia mostly occurs in older people, but can theoretically occur in any age group. Whereas acute leukemia must be treated immediately, chronic forms are sometimes monitored for some time before treatment to ensure maximum effectiveness of therapy.


Additionally, the diseases are subdivided according to which kind of blood cell is affected. This split divides leukemias into lymphoblastic or ''[[lymphocytic leukemia]]s'' and myeloid or ''[[myelogenous leukemia]]s'':
Leukemia may be classified as follows:
<br><br>
{{Family tree/start}}
{{Family tree | | | | | | | | | B01 | | | |B01= Leukemia}}
{{Family tree | | | | |,|-|-|-|-|^|-|-|-|-|.| | }}
{{Family tree | | | | C01 | | | | | | | | C02 |C01= Lymphoid progeny| C02= Myeloid progeny}}
{{Family tree | |,|-|-|^|-|-|.| | | |,|-|-|^|-|-|.| | }}
{{Family tree | D01 | | | | D02 | | D03 | | | | D04 | D01= [[Acute lymphoblastic leukemia]] (ALL) | D02= [[Chronic lymphocytic leukemia]] (CLL) | D03= [[Acute Myeloid Leukemia]] (AML) | D04= [[Chronic myeloid leukemia]] (CML)}}
{{Family tree/end}}
<br><br><br>


* In lymphoblastic or ''[[lymphocytic leukemia]]s'', the cancerous change takes place in a type of marrow cell that normally goes on to form [[lymphocyte]]s.
==Differentiating Leukemia from other Diseases==
* In myeloid or ''[[myelogenous leukemia]]s'', the cancerous change takes place in a [[myeloid cells|type of marrow cell]] that normally goes on to form red cells, some types of white cells, and [[platelets]].
Leukemia must be differentiated from various diseases that cause [[weight loss]],  [[night sweats]], [[hepatosplenomegaly]], and palpable [[lymph node]]s, such as [[hairy cell leukaemia|hairy cell leukemia]], prolymphocytic leukemia, [[follicular lymphoma]], and [[mantle cell lymphoma]]. Based on the expression of cell surface markers, the table below differentiates different types of leukemia from other diseases that cause similar clinical presentations:<ref name="H">Hoffbrand V, Moss P. Essential Haematology. John Wiley & Sons; 2011</ref>


Combining these two classifications provides a total of four main categories:
{|
! rowspan="2" align="center" style="background:#4479BA; color: #FFFFFF;" + |Disease
! rowspan="2" align="center" style="background:#4479BA; color: #FFFFFF;" + |Etiology
! colspan="4" align="center" style="background:#4479BA; color: #FFFFFF;" + |Clinical Manifestation
! colspan="2" align="center" style="background:#4479BA; color: #FFFFFF;" + |Laboratory Findings
! rowspan="2" align="center" style="background:#4479BA; color: #FFFFFF;" + |Gold standard diagnosis
! rowspan="2" align="center" style="background:#4479BA; color: #FFFFFF;" + |Associated findings
|-
! align="center" style="background:#4479BA; color: #FFFFFF;" + |Demography
! align="center" style="background:#4479BA; color: #FFFFFF;" + |History
! align="center" style="background:#4479BA; color: #FFFFFF;" + |Symptoms
! align="center" style="background:#4479BA; color: #FFFFFF;" + |Signs
! align="center" style="background:#4479BA; color: #FFFFFF;" + |Lab
! align="center" style="background:#4479BA; color: #FFFFFF;" + |Histopathology
|-
! align="center" style="background:#DCDCDC;" + |[[Acute myelogenous leukemia]]<ref name="pmid30410824">{{cite journal |vauthors=Saif A, Kazmi SFA, Naseem R, Shah H, Butt MO |title=Acute Myeloid Leukemia: Is That All There Is? |journal=Cureus |volume=10 |issue=8 |pages=e3198 |date=August 2018 |pmid=30410824 |doi=10.7759/cureus.3198 |url=}}</ref><ref name="pmid23526416">{{cite journal |vauthors=Estey EH |title=Acute myeloid leukemia: 2013 update on risk-stratification and management |journal=Am. J. Hematol. |volume=88 |issue=4 |pages=318–27 |date=April 2013 |pmid=23526416 |doi=10.1002/ajh.23404 |url=}}</ref>
| align="left" style="background:#F5F5F5;" + |
* Clonal proliferation of malignant myeloid blast cells in the [[bone marrow]]
* Genetic abnormalities t(8;21), inv(16), and t(15;17)
| align="left" style="background:#F5F5F5;" + |
* The most common leukemia in adults
* Median age of 63 years old
| align="left" style="background:#F5F5F5;" + |
* [[Smoking]], previous [[chemotherapy]] or [[radiation therapy]], [[myelodysplastic syndrome]], and exposure to the chemical [[benzene]]
| align="left" style="background:#F5F5F5;" + |
* [[Fatigue]]
* [[Bleeding]]
| align="left" style="background:#F5F5F5;" + |
* Bone [[tenderness]]
* [[Dyspnea]]
* Leukemia cutis
* Swelling of the [[Gingiva|gums]]
* Chloroma
* Rare LAP
| align="left" style="background:#F5F5F5;" + |
* [[Anemia]]
* [[Thrombocytopenia]]
* [[Leukocytosis]] or [[leukopenia]]
| align="left" style="background:#F5F5F5;" + |
* Leukemic blasts
* Positive [[Auer rod|Auer rods]]
| align="left" style="background:#F5F5F5;" + |
* [[Flow cytometry]] > 20% blasts of [[myeloid]] lineage
| align="left" style="background:#F5F5F5;" + |
* Persistent or frequent [[infections]]
* Fatal within weeks or months if left untreated
* [[Down syndrome]] or [[Bloom syndrome]]
|-
! align="center" style="background:#DCDCDC;" + |[[Acute lymphoblastic leukemia]]<ref name="pmid30302234">{{cite journal |vauthors=Sawalha Y, Advani AS |title=Management of older adults with acute lymphoblastic leukemia: challenges & current approaches |journal=Int J Hematol Oncol |volume=7 |issue=1 |pages=IJH02 |date=March 2018 |pmid=30302234 |pmc=6176956 |doi=10.2217/ijh-2017-0023 |url=}}</ref><ref name="pmid23841506">{{cite journal |vauthors=Portell CA, Advani AS |title=Novel targeted therapies in acute lymphoblastic leukemia |journal=Leuk. Lymphoma |volume=55 |issue=4 |pages=737–48 |date=April 2014 |pmid=23841506 |doi=10.3109/10428194.2013.823493 |url=}}</ref>
| align="left" style="background:#F5F5F5;" + |
* Arrest of  [[lymphoblasts]]
* Chromosomal translocations: t(9;22) , t(12;21), t(5;14), t(1;19)
| align="left" style="background:#F5F5F5;" + |
* The most common  cancer in children
*Peak 2-5 years of age
*Boys > girls
| align="left" style="background:#F5F5F5;" + |
* History of [[cancer]]
* History of drug exposure
| align="left" style="background:#F5F5F5;" + |
* Generalized [[weakness]]
* [[Fatigue]]
* [[Bleeding]]
| align="left" style="background:#F5F5F5;" + |
* [[Hepatosplenomegaly]]
* [[Lymphadenopathy|LAP]]
* [[Dyspnea]]
* [[Pallor]]
* [[Papilledema]]
* [[Meningism|Nuchal rigidity]]
* [[Cranial nerve palsy]]
* [[Testicle|Testicular]] enlargement
* [[Mediastinal mass]]
| align="left" style="background:#F5F5F5;" + |
* [[Anemia]]
* [[Thrombocytopenia]] 
* Normal or slightly increased [[White blood cells|WBC]] counts 
| align="left" style="background:#F5F5F5;" + |
* [[Lymphoblast|Lymphoblasts]]
* Atypical cells
| align="left" style="background:#F5F5F5;" + |
* [[Bone marrow examination|Bone marrow biopsy]]
| align="left" style="background:#F5F5F5;" + |
* [[CNS]] involvement
|-
! align="center" style="background:#DCDCDC;" + |[[Chronic myelogenous leukemia]]<ref name="pmid25814082">{{cite journal |vauthors=Saußele S, Silver RT |title=Management of chronic myeloid leukemia in blast crisis |journal=Ann. Hematol. |volume=94 Suppl 2 |issue= |pages=S159–65 |date=April 2015 |pmid=25814082 |doi=10.1007/s00277-015-2324-0 |url=}}</ref><ref name="pmid30285354">{{cite journal |vauthors=Eden RE, Coviello JM |title= |journal= |volume= |issue= |pages= |date= |pmid=30285354 |doi= |url=}}</ref>
| align="left" style="background:#F5F5F5;" + |
* Dysregulated production and uncontrolled proliferation of mature and maturing [[Granulocyte|granulocytes]]
* [[BCR/ABL|BCR-ABL1]] fusion gene
* [[Dominance relationship|Autosomal dominant]] mutation
| align="left" style="background:#F5F5F5;" + |
* Median age 50 years old
| align="center" style="background:#F5F5F5;" + |
| align="left" style="background:#F5F5F5;" + |
* Generalized weakness
* [[Fatigue]]
* [[Satiety|Early satiety]]
* Abdominal fullness
* Bleeding
| align="left" style="background:#F5F5F5;" + |
*Asymptomatic
*[[Blast crisis]]
*Excessive [[Perspiration|sweating]]
* [[Papilledema]]
* [[Tenderness]] over the lower [[sternum]]
* [[Hepatosplenomegaly]]
| align="left" style="background:#F5F5F5;" + |
* [[Anemia]]
* [[White blood cells|WBC]] > 100,000/microL
* Absolute [[basophilia]] and [[eosinophilia]]
* [[Platelet|Plt]] > 600,000 to 700,000/microL
* Low [[leukocyte alkaline phosphatase]]
* High [[uric acid]]
| align="left" style="background:#F5F5F5;" + |
* All cells of the neutrophilic series, from [[Myeloblast|myeloblasts]] to mature [[Neutrophil|neutrophils]]
* [[Myelocyte]] bulge
| align="left" style="background:#F5F5F5;" + |
* [[Bone marrow examination|Bone marrow biopsy]]
| align="left" style="background:#F5F5F5;" + |
* Acute [[Gout|gouty arthritis]]
* Venous obstruction
|-
! align="center" style="background:#4479BA; color: #FFFFFF;" + |Disease
! align="center" style="background:#4479BA; color: #FFFFFF;" + |Etiology
! align="center" style="background:#4479BA; color: #FFFFFF;" + |Demography
! align="center" style="background:#4479BA; color: #FFFFFF;" + |History
! align="center" style="background:#4479BA; color: #FFFFFF;" + |Symptoms
! align="center" style="background:#4479BA; color: #FFFFFF;" + |Signs
! align="center" style="background:#4479BA; color: #FFFFFF;" + |Lab
! align="center" style="background:#4479BA; color: #FFFFFF;" + |Histopathology
! align="center" style="background:#4479BA; color: #FFFFFF;" + |Gold standard diagnosis
! align="center" style="background:#4479BA; color: #FFFFFF;" + |Associated findings
|-
! align="center" style="background:#DCDCDC;" + |'''[[Chronic lymphocytic leukemia]]'''<ref name="pmid266906142">{{cite journal |vauthors=Rai KR, Jain P |title=Chronic lymphocytic leukemia (CLL)-Then and now |journal=Am. J. Hematol. |volume=91 |issue=3 |pages=330–40 |date=March 2016 |pmid=26690614 |doi=10.1002/ajh.24282 |url=}}</ref>
| align="left" style="background:#F5F5F5;" + |
* Progressive accumulation of monoclonal [[B cell|B lymphocytes]]
| align="left" style="background:#F5F5F5;" + |
* The most common leukemia in adults in western countries
* M > F
* Median age 70 years old
| align="left" style="background:#F5F5F5;" + |
* Positive family history
* Exposure to [[Herbicide|herbicides]] or [[Insecticide|insecticides]]
| align="left" style="background:#F5F5F5;" + |
* Bleeding
* Abdominal pain
* Generalized [[weakness]]
* [[Anorexia]]
| align="left" style="background:#F5F5F5;" + |
* LAP (Most common sign)
* [[Hepatosplenomegaly]]
* [[Skin lesion|Skin lesions]] (leukemia cutis)
* [[Sleep hyperhidrosis|Night sweats]]
* [[Muscle wasting]]
| align="left" style="background:#F5F5F5;" + |
* [[Anemia]]
* [[Thrombocytopenia]]
* Absolute [[lymphocytosis]] >5000 cells/μl
* [[Neutropenia]]
* Positive direct Coombs test
* [[Hypogammaglobulinemia]]
* Elevated [[lactate dehydrogenase]] and [[beta-2 microglobulin]]
| align="left" style="background:#F5F5F5;" + |
* Presence of smudge cells
* Monoclonality of [[Light chain|kappa]] and [[Lambda (anatomy)|lambda]] producing [[B cell|B cells]]
* Express [[CD19]], [[CD20]], [[CD23]], and [[CD5]] on the [[cell]] surface
| align="left" style="background:#F5F5F5;" + |
* [[Flow cytometry]] of the [[Venous blood|peripheral blood]]
| align="left" style="background:#F5F5F5;" + |
* Extranodal involvement of [[skin]], [[kidney]], [[lung]], [[Spinal cord|spine]]
* [[Membranoproliferative glomerulonephritis]]
* [[Autoimmune hemolytic anemia]]
|-
! align="center" style="background:#DCDCDC;" + |[[Hairy cell leukemia]]<ref name="pmid29110361">{{cite journal |vauthors=Troussard X, Cornet E |title=Hairy cell leukemia 2018: Update on diagnosis, risk-stratification, and treatment |journal=Am. J. Hematol. |volume=92 |issue=12 |pages=1382–1390 |date=December 2017 |pmid=29110361 |pmc=5698705 |doi=10.1002/ajh.24936 |url=}}</ref><ref name="pmid29118233">{{cite journal |vauthors=Wierda WG, Byrd JC, Abramson JS, Bhat S, Bociek G, Brander D, Brown J, Chanan-Khan A, Coutre SE, Davis RS, Fletcher CD, Hill B, Kahl BS, Kamdar M, Kaplan LD, Khan N, Kipps TJ, Lancet J, Ma S, Malek S, Mosse C, Shadman M, Siddiqi T, Stephens D, Wagner N, Zelenetz AD, Dwyer MA, Sundar H |title=Hairy Cell Leukemia, Version 2.2018, NCCN Clinical Practice Guidelines in Oncology |journal=J Natl Compr Canc Netw |volume=15 |issue=11 |pages=1414–1427 |date=November 2017 |pmid=29118233 |doi=10.6004/jnccn.2017.0165 |url=}}</ref>
| align="left" style="background:#F5F5F5;" + |
* Accumulation of small mature [[B cell]] lymphoid cells with abundant [[cytoplasm]] and "hairy" projections
* [[BRAF]] mutation
| align="left" style="background:#F5F5F5;" + |
* Uncommon
* Median age 50 to 55 years old
* M >> F
* More common in Caucasians than Blacks
| align="left" style="background:#F5F5F5;" + |
* Exposures to [[ionizing radiation]], [[Pesticide|pesticides]], and farming
| align="left" style="background:#F5F5F5;" + |
* Generalized weakness
* [[Fatigue]]
* [[Satiety|Early satiety]]
* Abdominal fullness
* Bleeding
| align="left" style="background:#F5F5F5;" + |
* Asymptomatic
* [[Splenomegaly]]
* Spontaneous [[splenic rupture]]
* [[Rash|Skin rash]]
* [[Ascites]]
* [[Pleural effusion]]
| align="left" style="background:#F5F5F5;" + |
* [[Cytopenia]]
* [[Leukocytosis]] in 10 to 20 percent
* [[Azotemia]]
* Abnormal [[liver function tests]]
* [[Hypergammaglobulinemia]]
| align="left" style="background:#F5F5F5;" + |
* [[Pancytopenia]] with [[monocytopenia]] and circulating tumor cells characteristic of HCL
* Dry [[bone marrow]]
| align="left" style="background:#F5F5F5;" + |
* Analysis of [[Venous blood|peripheral blood]] + [[immunophenotyping]] by [[flow cytometry]]
| align="left" style="background:#F5F5F5;" + |
* [[Vasculitis]]
|-
! align="center" style="background:#DCDCDC;" + |Large granular lymphocytic leukemia<ref name="pmid28128670">{{cite journal |vauthors=Matutes E |title=Large granular lymphocytic leukemia. Current diagnostic and therapeutic approaches and novel treatment options |journal=Expert Rev Hematol |volume=10 |issue=3 |pages=251–258 |date=March 2017 |pmid=28128670 |doi=10.1080/17474086.2017.1284585 |url=}}</ref><ref name="pmid28717070">{{cite journal |vauthors=Oshimi K |title=Clinical Features, Pathogenesis, and Treatment of Large Granular Lymphocyte Leukemias |journal=Intern. Med. |volume=56 |issue=14 |pages=1759–1769 |date=2017 |pmid=28717070 |pmc=5548667 |doi=10.2169/internalmedicine.56.8881 |url=}}</ref>​
| align="left" style="background:#F5F5F5;" + |
* Clonal proliferation of [[Cytotoxic T cell|cytotoxic T cells]]
* Dysregulation of [[apoptosis]] through abnormalities in the Fas/Fas ligand pathway
| align="left" style="background:#F5F5F5;" + |
* Rare
* Median age 60 years
* M = F
| align="left" style="background:#F5F5F5;" + |
* Autoimmune diseases
* [[Lymphoproliferative disorders|Lymphoproliferative]] disorders
| align="left" style="background:#F5F5F5;" + |
* Generalized [[weakness]]
* [[Fatigue]]
| align="left" style="background:#F5F5F5;" + |
* Mostly asymptomatic
| align="left" style="background:#F5F5F5;" + |
* Modest [[lymphocytosis]]
* [[Neutropenia]]
* [[Anemia]]
* [[Thrombocytopenia]]
| align="left" style="background:#F5F5F5;" + |
* Large [[Lymphocyte|lymphocytes]] with a condensed round or oval nucleus, abundant pale basophilic cytoplasm, and small azurophilic granules
| align="left" style="background:#F5F5F5;" + |
* Biopsy and flow cytometry + T-cell receptor gene rearrangement studies
| align="left" style="background:#F5F5F5;" + |
* Recurrent bacterial infection
|-
! align="center" style="background:#DCDCDC;" + |[[Chronic neutrophilic leukemia]]<ref name="pmid29512199">{{cite journal |vauthors=Elliott MA, Tefferi A |title=Chronic neutrophilic leukemia: 2018 update on diagnosis, molecular genetics and management |journal=Am. J. Hematol. |volume=93 |issue=4 |pages=578–587 |date=August 2018 |pmid=29512199 |doi=10.1002/ajh.24983 |url=}}</ref>
| align="left" style="background:#F5F5F5;" + |
* Mature granulocytic proliferation in the blood and [[bone marrow]]
* Point mutations in the [[CSF3R]] gene
| align="left" style="background:#F5F5F5;" + |
* Very rare
* M = F
| align="left" style="background:#F5F5F5;" + |
* [[Multiple myeloma]]
| align="left" style="background:#F5F5F5;" + |
* Generalized [[weakness]]
* [[Fatigue]]
| align="left" style="background:#F5F5F5;" + |
* [[Hepatosplenomegaly]]
* [[Pruritus]]
* [[Gout]]
| align="left" style="background:#F5F5F5;" + |
* Peripheral blood [[neutrophilia]] (> 25 x 10<sup>9</sup>/L) with myeloid precursors (promyelocytes, myelocytes, metamyelocytes)
* Elevated [[leukocyte alkaline phosphatase]]
| align="left" style="background:#F5F5F5;" + |
* Toxic granulation in the [[Neutrophil|neutrophils]]
* Nuclear hypersegmentation
* Increased myeloid:erythroid ratio > 20:1
| align="left" style="background:#F5F5F5;" + |
* [[World Health Organization|WHO]] diagnostic criteria include leukocytosis of ≥ 25 x 109/L
* More than 80% neutrophils,
* Less than 10% circulating neutrophil precursors with blasts
| align="left" style="background:#F5F5F5;" + |
* Poor prognosis
* Absence of the Philadelphia chromosome or a BCR/ABL fusion gene
|-
! align="center" style="background:#4479BA; color: #FFFFFF;" + |Disease
! align="center" style="background:#4479BA; color: #FFFFFF;" + |Etiology
! align="center" style="background:#4479BA; color: #FFFFFF;" + |Demography
! align="center" style="background:#4479BA; color: #FFFFFF;" + |History
! align="center" style="background:#4479BA; color: #FFFFFF;" + |Symptoms
! align="center" style="background:#4479BA; color: #FFFFFF;" + |Signs
! align="center" style="background:#4479BA; color: #FFFFFF;" + |Lab
! align="center" style="background:#4479BA; color: #FFFFFF;" + |Histopathology
! align="center" style="background:#4479BA; color: #FFFFFF;" + |Gold standard diagnosis
! align="center" style="background:#4479BA; color: #FFFFFF;" + |Associated findings
|-
! align="center" style="background:#DCDCDC;" + |[[Chronic eosinophilic leukemia]]
| align="left" style="background:#F5F5F5;" + |
* There is no known cause for chronic eosinophilic leukemia.
* It hasn't been linked to a specific chromosome or genetic abnormality.
| align="left" style="background:#F5F5F5;" + |
* Unknown
| align="left" style="background:#F5F5F5;" + |
* Unknown
| align="left" style="background:#F5F5F5;" + |
* Constitutional
* [[Rash]]
* [[Rhinitis]]
* [[Gastritis]]
* [[Thromboembolism]]<nowiki/>related
| align="left" style="background:#F5F5F5;" + |
* [[Hypertension]]
* [[Eczema]], [[mucosal]][[ulcers]], [[erythema]]
* [[Angioedema]]


{| class="wikitable"
* [[Ataxia]]
|+Four major kinds of leukemia
* [[Anemia]]
! Cell type !! Acute !! Chronic
* [[Lymphadenopathy]]
|-
* [[Hepatosplenomegaly]]
| '''Lymphocytic leukemia''' <br />(or "lymphoblastic") || [[Acute lymphoblastic leukemia]] (ALL) || [[Chronic lymphocytic leukemia]] (CLL)
| align="left" style="background:#F5F5F5;" + |
|-
*[[Leukocytosis]] with left shift
| '''Myelogenous leukemia''' <br />(also "myeloid" or "nonlymphocytic") || [[Acute myeloid leukemia|Acute myelogenous leukemia]] (AML) || [[Chronic myelogenous leukemia]] (CML)
*[[Eosinophilia]]
|}
*[[Basophilia]]
*[[Monocytosis]]
*[[Anemia]]
*[[Thrombocytopenia]]
*↑ [[B12]] levels
*↑ [[LDH]]


Within these main categories, there are typically several subcategories. Finally, [[hairy cell leukemia]] is usually considered to be outside of this classification scheme.
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* Hypercelluar with ↑ [[eosinophilic]]<nowiki/> precursors, ↑ [[eosinophils]], and atypical [[mononuclear cells]]
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*[[Heart failure]] [[Lung fibrosis]]
*[[Encephalopathy]]
*Erythema annulare centrifugam
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! align="center" style="background:#DCDCDC;" + |[[Chronic monocytic leukemia electrocardiogram|Chronic monocytic leukemia]]
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! align="center" style="background:#DCDCDC;" + |[[Prolymphocytic leukemia]] (PLL)
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! align="center" style="background:#DCDCDC;" + |[[T-cell large granular lymphocyte leukemia|T-cell large granular lymphocytic leukemia]] (TLGL)
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! align="center" style="background:#4479BA; color: #FFFFFF;" + |Disease
! align="center" style="background:#4479BA; color: #FFFFFF;" + |Etiology
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! align="center" style="background:#4479BA; color: #FFFFFF;" + |Histopathology
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! align="center" style="background:#4479BA; color: #FFFFFF;" + |Associated findings
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! align="center" style="background:#DCDCDC;" + |[[Aggressive NK-cell leukemia]] (ANKL)
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! align="center" style="background:#DCDCDC;" + |[[Adult T-cell leukemia|Adult T-cell leukemia/lymphoma]] (ATLL)<ref name="wiki1">Human T-lymphotropic virus. Wikipedia (2015) https://en.wikipedia.org/wiki/Human_T-lymphotropic_virus#Transmission Accessed on November, 3 2015</ref><ref name="pmid18042693">{{cite journal |vauthors=Matutes E |title=Adult T-cell leukaemia/lymphoma |journal=J. Clin. Pathol. |volume=60 |issue=12 |pages=1373–7 |year=2007 |pmid=18042693 |pmc=2095573 |doi=10.1136/jcp.2007.052456 |url=}}</ref><ref name="wiki">Adult T-cell leukemia/lymphoma. Wikipedia (2015) https://en.wikipedia.org/wiki/Adult_T-cell_leukemia/lymphoma Accessed on November, 3 2015</ref><ref name="pmid20425378">{{cite journal| author=Mahieux R, Gessain A| title=Adult T-cell leukemia/lymphoma and HTLV-1. | journal=Curr Hematol Malig Rep | year= 2007 | volume= 2 | issue= 4 | pages= 257-64 | pmid=20425378 | doi=10.1007/s11899-007-0035-x | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=20425378  }}</ref><ref name="wiki2">Adult T-cell leukemia/lymphoma. Wikipedia (2015) https://en.wikipedia.org/wiki/Adult_T-cell_leukemia/lymphoma Accessed on November, 3 2015</ref><ref name="pmid180426932">{{cite journal| author=Matutes E| title=Adult T-cell leukaemia/lymphoma. | journal=J Clin Pathol | year= 2007 | volume= 60 | issue= 12 | pages= 1373-7 | pmid=18042693 | doi=10.1136/jcp.2007.052456 | pmc=PMC2095573 | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=18042693  }}</ref>
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* Adult T-cell leukemia is caused by an infection with [[HTLV]].
* Common [[genetic mutation]]s involved in the development of adult T-cell leukemia can be found [[Adult T-cell leukemia pathophysiology|here]].


* ''Acute lymphoblastic leukemia'' (ALL) is the most common type of leukemia in young children. This disease also affects adults, especially those age 65 and older. Standard treatments involve chemotherapy and radiation. The survival rates vary by age: 85% in children and 50% in adults.<ref>[http://www.accessmedicine.com/content.aspx?aID=65842 Harrison's Principles of Internal Medicine, 16th Edition,] Chapter 97. Malignancies of Lymphoid Cells. Clinical Features, Treatment, and Prognosis of Specific Lymphoid Malignancies.</ref>  
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* The incidence of adult T-cell leukemia increases with age, and the median age at diagnosis is 57 years.
* Males are more commonly affected with adult T-cell leukemia than females.
* The male to female ratio is approximately 1.4 to 1.
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:* [[Abdominal pain]]
:* [[Constipation]]
:* [[Nausea]] and [[vomiting]]
:* [[Fatigue]]
:* Generalized [[weakness]]
:* [[Cough]]
:* Recurrent [[infection]]s
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:* [[Fever]]
:* [[Weight loss]]
:* Recurrent [[bleeding]]
:* [[Anorexia]]
:* [[Night sweat]]s
:* [[Bone pain]]
:* [[Hypercalcemia]]
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! align="center" style="background:#DCDCDC;" + |[[Sezary syndrome]]<ref name="pmid21883142">{{cite journal |vauthors=Wong HK, Mishra A, Hake T, Porcu P |title=Evolving insights in the pathogenesis and therapy of cutaneous T-cell lymphoma (mycosis fungoides and Sezary syndrome) |journal=Br. J. Haematol. |volume=155 |issue=2 |pages=150–66 |date=October 2011 |pmid=21883142 |pmc=4309373 |doi=10.1111/j.1365-2141.2011.08852.x |url=}}</ref><ref name="pmid27121473">{{cite journal |vauthors=Woollard WJ, Pullabhatla V, Lorenc A, Patel VM, Butler RM, Bayega A, Begum N, Bakr F, Dedhia K, Fisher J, Aguilar-Duran S, Flanagan C, Ghasemi AA, Hoffmann RM, Castillo-Mosquera N, Nuttall EA, Paul A, Roberts CA, Solomonidis EG, Tarrant R, Yoxall A, Beyers CZ, Ferreira S, Tosi I, Simpson MA, de Rinaldis E, Mitchell TJ, Whittaker SJ |title=Candidate driver genes involved in genome maintenance and DNA repair in Sézary syndrome |journal=Blood |volume=127 |issue=26 |pages=3387–97 |date=June 2016 |pmid=27121473 |doi=10.1182/blood-2016-02-699843 |url=}}</ref><ref name="pmid266071833">{{cite journal |vauthors=Wilcox RA |title=Cutaneous T-cell lymphoma: 2016 update on diagnosis, risk-stratification, and management |journal=Am. J. Hematol. |volume=91 |issue=1 |pages=151–65 |date=January 2016 |pmid=26607183 |pmc=4715621 |doi=10.1002/ajh.24233 |url=}}</ref><ref name="pmid25386354">{{cite journal |vauthors=Horesh N, Horowitz NA |title=Does gender matter in non-hodgkin lymphoma? Differences in epidemiology, clinical behavior, and therapy |journal=Rambam Maimonides Med J |volume=5 |issue=4 |pages=e0038 |date=October 2014 |pmid=25386354 |pmc=4222427 |doi=10.5041/RMMJ.10172 |url=}}</ref><ref name="pmid24421750">{{cite journal |vauthors=Al Hothali GI |title=Review of the treatment of mycosis fungoides and Sézary syndrome: A stage-based approach |journal=Int J Health Sci (Qassim) |volume=7 |issue=2 |pages=220–39 |date=June 2013 |pmid=24421750 |pmc=3883611 |doi= |url=}}</ref><ref name="pmid266071835">{{cite journal |vauthors=Wilcox RA |title=Cutaneous T-cell lymphoma: 2016 update on diagnosis, risk-stratification, and management |journal=Am. J. Hematol. |volume=91 |issue=1 |pages=151–65 |date=January 2016 |pmid=26607183 |pmc=4715621 |doi=10.1002/ajh.24233 |url=}}</ref><ref name="pmid231971992">{{cite journal |vauthors=Yamashita T, Abbade LP, Marques ME, Marques SA |title=Mycosis fungoides and Sézary syndrome: clinical, histopathological and immunohistochemical review and update |journal=An Bras Dermatol |volume=87 |issue=6 |pages=817–28; quiz 829–30 |date=2012 |pmid=23197199 |pmc=3699909 |doi= |url=}}</ref><ref name="pmid23074497">{{cite journal |vauthors= |title=Extracorporeal photophoresis: an evidence-based analysis |journal=Ont Health Technol Assess Ser |volume=6 |issue=6 |pages=1–82 |date=2006 |pmid=23074497 |pmc=3379535 |doi= |url=}}</ref>
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* The cause of Sezary syndrome has not been identified.
* Sezary syndrome might have one or more of the [[chromosomal]] [[abnormalities]], such as the loss or gain of [[Genetics|genetic]].
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* The prevalence of Sezary syndrome is exact unknown.
*The median age at diagnosis of [[Sézary syndrome]] is 60 years of age.
*Sezary syndrome is more commonly observed among [[Old age|elderly]] [[Patient|patients]].
*[[Male|Males]] are more commonly affected with Sezary syndrome than [[Female|females]](2:1).
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*The majority of sezary syndrome [[Patient|patients]] present with developing [[lymphadenopathy]] and [[erythroderma]] for weeks to months.
*Early clinical features of Sezary syndrome include:
:*Mimic [[psoriasis]]
:*[[Chronic (medical)|Chronic]] [[eczema]]
:*[[Atopic dermatitis (patient information)|Atopic dermatitis]]
:*[[Leprosy]]
:*Lichenoid [[pityriasis]]
*In Sezary syndrome single or multiple [[Lesion|lesions]] (thin [[erythematous]] [[Plaque|plaques]] or flat patch) is a typical [[skin]] involvement  in the [[gluteal]] region or [[Thigh|thighs]]. <ref name="Olsen2015">{{cite journal|last1=Olsen|first1=Elise A.|title=Evaluation, Diagnosis, and Staging of Cutaneous Lymphoma|journal=Dermatologic Clinics|volume=33|issue=4|year=2015|pages=643–654|issn=07338635|doi=10.1016/j.det.2015.06.001}}</ref>
*[[Patient|Patients]] with Sezary syndrome  often have a history of several years of [[Eczema|eczematous]] or [[dermatitis]] [[skin]] [[Lesion|lesions]] before the [[diagnosis]] is finally established.
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* Widespread [[erythema]]
** In Sezary syndrome widespread [[erythema]] can be finely scaly, indurated, or even resemble livido [[reticularis]]
* Indurated
* Resemble livido [[reticularis]]
* [[Erythema]](Not seen in some [[Patient|patients]])
* The severity of [[erythema]] [[body surface area]] (BSA) involved may wax and wane(>80% of BSA)


* ''Chronic lymphocytic leukemia'' (CLL) most often affects adults over the age of 55. It sometimes occurs in younger adults, but it almost never affects children. Two-thirds of affected people are men. The five-year survival rate is 75%.<ref>[http://seer.cancer.gov/statfacts/html/clyl.html Finding Cancer Statistics » Cancer Stat Fact Sheets »Chronic Lymphocytic Leukemia] National Cancer Institute</ref> It is incurable, but there are many effective treatments.
* Patches and [[Plaque|plaques]] to [[erythroderma]]
* [[Keratosis pilaris]]
* [[Alopecia]] ([[hair loss]])
* [[Ectropion]]
* [[Keratoderma]]
* [[Hypertrophy (medical)|Hypertrophic]] [[Nail (anatomy)|nails]]
* Erosions
* [[Lichenification]]
* [[Trouble]] regulating [[Body Temperature|body temperature]]
* [[Abnormal|Abnormalities]] of [[fingernails]] and [[toenails]]


* ''Acute myelogenous leukemia'' (AML) occurs more commonly in adults than in children, and more commonly in men than women.  AML is treated with chemotherapy.  The five-year survival rate is 40%.<ref>{{cite journal |author=Colvin GA, Elfenbein GJ |title=The latest treatment advances for acute myelogenous leukemia |journal=Med Health R I |volume=86 |issue=8 |pages=243–6 |year=2003 |pmid=14582219 |doi=}}</ref>
* [[Lymphadenopathy]]
* [[Viscera|Viscer]]
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! align="center" style="background:#DCDCDC;" + |[[Myelodysplastic syndrome]]
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*Constitutional symptoms
*[[Bleeding]]
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*[[Pallor]]
*[[Petechiae]]
*[[Organomegaly]]
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* [[Pancytopenia]]
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*Hypercellular/ normocellular [[bone marrow]] with [[Dysplastic change|dysplastic]] changes
*Macro-ovalocytes
*Basophilic stippling
*[[Howell-Jolly body]]
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*[[Leukemia]] transformation
*Acquired pseudo-Pelger-Huët anomaly
*Infection
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! align="center" style="background:#DCDCDC;" + |[[Myeloproliferative disorders]]
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! align="center" style="background:#DCDCDC;" + |[[Leukemoid reaction]]
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* ''Chronic myelogenous leukemia'' (CML) occurs mainly in adults.  A very small number of children also develop this disease.  Treatment is with [[imatinib]] (Gleevec) or other drugs.  The five-year survival rate is 90%.<ref>[http://www.medscape.com/viewarticle/536049 Patients with Chronic Myelogenous Leukemia Continue to Do Well on Imatinib at 5-Year Follow-Up] Medscape Medical News 2006</ref><ref>[http://professional.cancerconsultants.com/conference_asco_2006.aspx?id=37519 Updated Results of Tyrosine Kinase Inhibitors in CML] ASCO 2006 Conference Summaries</ref>
==Epidemiology and Demographics==
===Prevalence===


* ''Hairy cell leukemia'' (HCL) is sometimes considered a subset of CLL, but does not fit neatly into this pattern.  About 80% of affected people are adult men.  There are no reported cases in young children.  HCL is incurable, but easily treatable. Survival is 96% to 100% at ten years.<ref name="pmid16245328">{{cite journal |author=Else M, Ruchlemer R, Osuji N, ''et al'' |title=Long remissions in hairy cell leukemia with purine analogs: a report of 219 patients with a median follow-up of 12.5 years |journal=[[Cancer]] |volume=104 |issue=11 |pages=2442–8 |year=2005 |pmid=16245328 |doi=10.1002/cncr.21447}}</ref>
* In the United States, the age-adjusted [[prevalence]] of leukemia is 75.3 per 100,000 in 2011.<ref name="SEER">Howlader N, Noone AM, Krapcho M, Garshell J, Miller D, Altekruse SF, Kosary CL, Yu M, Ruhl J, Tatalovich Z,Mariotto A, Lewis DR, Chen HS, Feuer EJ, Cronin KA (eds). SEER Cancer Statistics Review, 1975-2011, National Cancer Institute. Bethesda, MD, http://seer.cancer.gov/csr/1975_2011/, based on November 2013 SEER data submission, posted to the SEER web site, April 2014.</ref>


==Causes and risk factors==
===Incidence===
There is no single known cause for all of the different types of leukemia. The different leukemias likely have different causes, and very little is certain about what causes them. Researchers have strong suspicions about four possible causes:


* natural or artificial ionizing radiation
* The delay-adjusted [[incidence]] of leukemia in 2011 was estimated as 15.48 per 100,000 individuals in the United States.
* certain kinds of chemicals
* some viruses
* genetic predispositions


Leukemia, like other cancers, result from [[somatic mutation]]s in the [[DNA]] which activate [[oncogene]]s or deactivate [[tumor suppressor gene]]s, and disrupt the regulation of cell death, differentiation or division. These mutations may occur spontaneously or as a result of exposure to [[ionizing radiation|radiation]] or [[carcinogen]]ic substances and are likely to be influenced by genetic factors.  Cohort and case-control studies have linked exposure to [[petrochemicals]], such as [[benzene]], and hair dyes to the development of some forms of leukemia.
* In 2011, the age-adjusted incidence of leukemia was 13.66 per 100,000 individuals in the United States.


[[virus (biology)|Viruses]] have also been linked to some forms of leukemia.  For example, certain cases of ALL are associated with viral infections by either the [[human immunodeficiency virus]] (HIV, responsible for [[AIDS]]) or [[human T-lymphotropic virus]] (HTLV-1 and -2, causing [[adult T-cell leukemia/lymphoma]]).
===Age===


[[Fanconi anemia]] is also a risk factor for developing [[Acute myeloid leukemia|acute myelogenous leukemia]].
* The overall age-adjusted incidence of leukemia in the United States between 2007 and 2011 was 13 per 100,000 occurrences. The age-adjusted incidence of leukemia by age category is:
** Under 65 years: 6.5 per 100,000
** 65 and over: 57.9 per 100,000


Until the cause or causes of leukemia are found, there is no way to prevent the disease.  Even when the causes become known, they may prove to be things which are not readily controllable, such as naturally occurring background radiation, and therefore not especially helpful for prevention purposes.
* Shown below is a table depicting the overall age-adjusted incidence of leukemia per 100,000 individuals by age in the United States between 2007 and 2011 for the different types of leukemia.


== Treatment options for leukemia by type ==
{| style="cellpadding=0; cellspacing= 0; width: 600px;"
|-
===Acute Myelogenous Leukemia (AML)===
| style="padding: 0 5px; font-size: 100%; background: #4682B4; color: #FFFFFF; width: 10%" align="center" | || style="padding: 0 5px; font-size: 100%; background: #4682B4; color: #FFFFFF; width: 10%" align="center" |'''Acute lymphoblastic leukemia'''|| style="padding: 0 5px; font-size: 100%; background: #4682B4; color: #FFFFFF; width: 10%" align="center" | '''Chronic lymphocytic leukemia'''|| style="padding: 0 5px; font-size: 100%; background: #4682B4; color: #FFFFFF; width: 10%" align="center" |'''Acute myeloid leukemia'''|| style="padding: 0 5px; font-size: 100%; background: #4682B4; color: #FFFFFF; width: 10%" align="center" |'''Chronic myeloid leukemia'''
It is most common for adults; more men than women are affected.
|-
Many different chemotherapeutic plans are available for the treatment of AML. Overall, the strategy is to control bone marrow and systemic (whole-body) disease while offering specific treatment for the central nervous system (CNS), if involved. In general, most oncologists rely on combinations of drugs for the initial, induction phase of chemotherapy. Such combination chemotherapy usually offers the benefits of early remission (lessening of the disease) and a lower risk of disease resistance. Consolidation or "maintenance" treatments may be given to prevent disease recurrence once remission has been achieved. Consolidation treatment often entails a repetition of induction chemotherapy or the intensification chemotherapy with added drugs. By contrast, maintenance treatment involves drug doses that are lower than those administered during the induction phase.  
| style="padding: 0 5px; font-size: 100%; background: #4682B4; color: #FFFFFF; width: 10%" align="center" |'''All ages'''|| style="font-size: 100; padding: 0 5px; background: #B8B8B8" align="left" | 1.7 || style="font-size: 100; padding: 0 5px; background: #B8B8B8" align="left" |4.4|| style="font-size: 100; padding: 0 5px; background: #B8B8B8" align="left" |3.8 || style="font-size: 100; padding: 0 5px; background: #B8B8B8" align="left" |1.7
|-
| style="padding: 0 5px; font-size: 100%; background: #4682B4; color: #FFFFFF; width: 10%" align="center" |'''<65''' || style="font-size: 100; padding: 0 5px; background: #B8B8B8" align="left" |1.7 || style="font-size: 100; padding: 0 5px; background: #B8B8B8" align="left" |1.4|| style="font-size: 100; padding: 0 5px; background: #B8B8B8" align="left" | 1.8 || style="font-size: 100; padding: 0 5px; background: #B8B8B8" align="left" |0.9
|-
| style="padding: 0 5px; font-size: 100%; background: #4682B4; color: #FFFFFF; width: 10%" align="center" |'''≥65''' || style="font-size: 100; padding: 0 5px; background: #B8B8B8" align="left" | 1.6 || style="font-size: 100; padding: 0 5px; background: #B8B8B8" align="left" |25.2 || style="font-size: 100; padding: 0 5px; background: #B8B8B8" align="left" |17.5|| style="font-size: 100; padding: 0 5px; background: #B8B8B8" align="left" |6.8
|}


In addition, specific treatment plans may be used, depending on the type of leukemia that has been diagnosed. Whatever the plan, it is important for the patient to understand the treatment that is being given and the decision-making process behind the choice.
===Gender===


====Initial treatment of AML====
* In the United States, the age-adjusted prevalence of leukemia by gender in 2011 was:
Initial treatment of AML usually begins with induction chemotherapy using a combination of drugs such as [[daunorubicin]] ([[DNR]]), [[cytarabine]] ([[ara-C]]), [[idarubicin]], [[thioguanine]], [[etoposide]], or [[mitoxantrone]], anabolic [[steroids]].
** In males: 92.7 per 100,000
** In females: 60.7 per 100,000
* In the United States, the delay-adjusted incidence of leukemia by gender in 2011 was:
** In males: 19.93 per 100,000 persons
** In females: 11.89 per 100,000 persons


====Follow-up treatment====
* In the United States, the age-adjusted incidence of leukemia by gender on 2011 was:
Follow-up therapy for such patients may involve:  
** In males: 17.58 per 100,000 persons
** In females: 10.49 per 100,000 persons


* supportive care, such as intravenous nutrition and treatment with oral antibiotics (e.g., ofloxacin, rifampin), especially in patients who have prolonged granulocytopenia; that is too few mature granulocytes (neutrophils), the bacteria-destroying white blood cells that contain small particles, or granules (< 100 granulocytes per cubic millimeter for 2 weeks)
* Shown below is an image depicting the delay-adjusted incidence and observed incidence of leukemia by gender and race in the United States between 1975 and 2011. These graphs were gathered from [[SEER]]: The Surveillance, Epidemiology, and End Results Program of the National Cancer Institute.
* injection with colony-stimulating factors such as granulocyte colony-stimulating factor (G-CSF), which may help to shorten the period of granulocytopenia that results from induction therapy
* transfusions with red blood cells and platelets


Patients with newly diagnosed disease also may be considered for stem cell transplantation (SCT), either from the bone marrow or other sources. Allogeneic bone marrow transplant (alloBMT) is reserved primarily for patients under 55 years of age who have a compatible family donor. Approximately half of newly diagnosed AML patients are in this age group, with 75% achieving a complete remission (CR) after induction and consolidation therapy. Allogeneic bone marrow transplant is available for about 15% of all patients with AML. Unfortunately, it is estimated that only 7% of all AML patients will be cured using this procedure.
[[Image:Incidence of leukemia by gender and race in USA.PNG|700px|thumb|These graphs are adapted from [[SEER]]: The Surveillance, Epidemiology, and End Results Program of the National Cancer Institute. - Delay-adjusted [[incidence]] and observed [[incidence]] of leukemia by gender and race in the United States between 1975 and 2011]]


People who receive stem cell transplantation (SCT, alloBMT) require protective isolation in the hospital, including filtered air, sterile food, and sterilization of the microorganisms in the gut, until their total white blood cell (WBC) count is above 500.
===Race===


Treatment of central nervous system leukemia, if present, may involve injection of chemotherapeutic drugs (e.g., cytarabine or ara-C, methotrexate) into the areas around the brain and spinal cord.
* Shown below is a table depicting the age-adjusted prevalence of leukemia by race in 2011 in the United States.


====Consolidation or maintenance therapy====
{| style="cellpadding=0; cellspacing= 0; width: 600px;"
Once the patient is in remission, he or she will receive consolidation or maintenance therapy, for example, consolidation therapy with high-dose ara-C (HDAC) with/without anthracycline drugs).  
|-
| style="padding: 0 5px; font-size: 100%; background: #4682B4; color: #FFFFFF; width: 10%" align="center" | || style="padding: 0 5px; font-size: 100%; background: #4682B4; color: #FFFFFF; width: 10%" align="center" |'''All Races''' || style="padding: 0 5px; font-size: 100%; background: #4682B4; color: #FFFFFF; width: 10%" align="center" |'''White''' || style="padding: 0 5px; font-size: 100%; background: #4682B4; color: #FFFFFF; width: 10%" align="center" |'''Black''' || style="padding: 0 5px; font-size: 100%; background: #4682B4; color: #FFFFFF; width: 10%" align="center" |'''Asian/Pacific Islander'''  || style="padding: 0 5px; font-size: 100%; background: #4682B4; color: #FFFFFF; width: 10%" align="center" |'''Hispanic'''
|-
| style="padding: 0 5px; font-size: 100%; background: #4682B4; color: #FFFFFF; width: 10%" align="center" |'''Age-adjusted prevalence'''|| style="font-size: 100; padding: 0 5px; background: #B8B8B8" align="left" |75.3 per 100,000|| style="font-size: 100; padding: 0 5px; background: #B8B8B8" align="left" |83.5 per 100,000|| style="font-size: 100; padding: 0 5px; background: #B8B8B8" align="left" |45.9 per 100,000|| style="font-size: 100; padding: 0 5px; background: #B8B8B8" align="left" |41.2 per 100,000|| style="font-size: 100; padding: 0 5px; background: #B8B8B8" align="left" |57.1 per 100,000
|}


If, however, the AML patient has resistant disease (about 15%) or relapses (about 70%), second remissions sometimes are achieved by treating them with:
* Shown below is an image depicting the incidence of leukemia by race in the United States between 1975 and 2011.


* conventional induction chemotherapy
[[Image:Incidence of leukemia by race in USA.PNG|Incidence of leukemia by race in the United States between 1975 and 2011]]
* high-dose ara-C (HDAC), with/without other drugs
* etoposide or other single chemotherapeutic agents


Elderly AML patients have special treatment concerns. They may be less able to tolerate the septicemia (blood poisoning) associated with granulocytopenia, and they often have higher rates of myelodysplastic ('preleukemia') syndrome (MDS). Individuals who are over age 75 or who have significant medical conditions can be treated effectively with low-dose ara-C. High-dose post-induction chemotherapy is unlikely to be tolerated by elderly patients.
<small> API: Asian/Pacific Islander; AI/AN: American Indian/ Alaska Native</small>


Until recently, the treatment plans and responses of children with AML did not differ much from those of adults. Yet new, more intensive induction and consolidation treatments have resulted in higher remission rates and prolonged survivals. Many induction trials have produced good results using combinations of cytarabine (ara-C) plus an anthracycline (e.g., daunorubicin, doxorubicin). In children under 3 years of age, the anthracycline used for induction should be chosen with care, since doxorubicin produces more toxicity and related deaths than daunorubicin.
==Prognosis==
===5-Year Survival===


Consolidation therapy is complex, but it should include at least two courses of high-dose ara-C (HDAC). Children who have hyperleukocytosis (too many white blood cells), especially monocytic M5 leukemia, have a poor prognosis.
* Between 2004 and 2010, the 5-year relative survival of patients with leukemia was 60.3%.


===Chronic Myelogenous Leukemia (CML)===
* When stratified by age, the 5-year relative survival of patients with leukemia was 68.5% (44.1% for patients <65 and ≥ 65 years of age respectively).
The challenge of treating newly diagnosed CML is to determine the best overall strategy to control the disease. General strategies for management include a variety of options:


'''Leukapheresis''', also known as a peripheral blood stem cell transplant, with stem cell cryopreservation (frozen storage) prior to any other treatment. The patient's blood is passed through a machine that removes the stem cells and then returns the blood to the patient. Leukapheresis usually takes 3 or 4 hours to complete. The stem cells may or may not be treated with drugs to kill any cancer cells. The stem cells then are stored until they are transplanted back into the patient.  
* Shown below is a table depicting the 5-year relative survival of patients by the type of leukemia in the United States between 2004 and 2010.


'''HLA (human leukocyte antigen) typing''' of all patients under age 60, as well as typing of siblings, parents, and children, if available. This procedure will determine whether a compatible donor is available for stem cell transplantation.
{| style="cellpadding=0; cellspacing= 0; width: 800px;"
 
|-
'''Pre-treatment fertility measures''' (e.g., cryopreservation of semen prior to treatment; completion of a pregnancy prior to treatment) in young patients who have not completed their families.
| style="padding: 0 5px; font-size: 100%; background: #4682B4; color: #FFFFFF; width: 10%" align="center" | || style="padding: 0 5px; font-size: 100%; background: #4682B4; color: #FFFFFF; width: 10%" align="center" |'''Acute lymphoblastic leukemia'''|| style="padding: 0 5px; font-size: 100%; background: #4682B4; color: #FFFFFF; width: 10%" align="center" | '''Chronic lymphocytic leukemia'''|| style="padding: 0 5px; font-size: 100%; background: #4682B4; color: #FFFFFF; width: 10%" align="center" |'''Acute myeloid leukemia'''|| style="padding: 0 5px; font-size: 100%; background: #4682B4; color: #FFFFFF; width: 10%" align="center" |'''Chronic myeloid leukemia'''
 
|-
''Interferon-alpha (INF-a) therapy'''.
| style="padding: 0 5px; font-size: 100%; background: #4682B4; color: #FFFFFF; width: 10%" align="center" |'''5-year survival'''|| style="font-size: 100; padding: 0 5px; background: #B8B8B8" align="center" | 70% || style="font-size: 100; padding: 0 5px; background: #B8B8B8" align="center" |83.5%|| style="font-size: 100; padding: 0 5px; background: #B8B8B8" align="center" |25.4% || style="font-size: 100; padding: 0 5px; background: #B8B8B8" align="center" |59.9%
 
|}
'''Chemotherapy''' with drugs such as hydroxyurea (Hydrea®), busulfan (Myleran®) or imatinib mesylate ([[Gleevec]](tm)).
 
In general, CML treatment options are divided into two groups: those that do not increase survival and those that do. Chemotherapeutic drugs such as hydroxyurea (Hydrea®) and busulfan (Myleran®) can normalize the blood count for a period of time, but they do not increase survival. They often are used to control blood counts in patients who cannot undergo SCT or who do not respond to interferon therapy because of age or medical considerations.
 
[[Gleevec]], is one of a new class of cancer drugs that disables an abnormal enzyme in the cancerous cell, kills it, but leaves healthy cells virtually untouched. Other cancer therapies, such as chemotherapy, attack healthy cells as well as cancer cells, leaving patients with unpleasant and often severe side effects.
 
In June of 2006, the Food and Drug Administration (FDA) approved the oral tyrosine kinase inhibitor dasatinib (Sprycel(tm)) to treat CML that does not respond to other therapy.
 
One treatment that does impact on CML survival is allogeneic bone marrow transplantation, the use of high dose chemotherapy and radiation followed by infusion of a donor bone marrow. This procedure removes the chromosomal abnormality in a large percentage of patients and for them is curative. In addition, there is treatment with interferon (INF). About 20% to 30% of patients taking interferon show elimination of the abnormal chromosome and improved survival. Recent findings also suggest that low-dose cytarabine (ara-C), in combination with interferon, may be more beneficial than interferon alone. For patients who do not respond to interferon, autologous or allogeneic stem cell transplantation is the only alternative.
 
Patients with advanced-phase disease may be treated with cytotoxic drugs. For example, individuals showing myeloid transformation may be given drugs that are used to induce remission in AML - that is, daunorubicin and cytarabine, with or without 6-thioguanine or etoposide. Blast cell numbers will be reduced temporarily, but they will increase again within 3 to 6 weeks. Individuals showing lymphoid transformation have a slightly better outlook. They are treated with drugs used in the management of acute lymphocytic leukemia (ALL) - that is, prednisone, vincristine, and daunorubicin, with or without L-asparaginase.
 
New drugs that are being studied in clinical trials of CML include homoherringtonine with interferon-alpha (INF-a), paclitaxel (Taxol®), QS21 (a plant extract that heightens immune responses), and amifostin (a chemical that lessens some side effects of chemotherapy). In addition, clinical trials are evaluating the potential benefits of substances such as vaccines, monoclonal antibodies (immunologic substances that can direct the patient's immune system to kill cancer cells), and hormones (e.g., growth factors, interleukins).
 
===Acute Lymphocytic Leukemia (ALL)===
Proper management of ALL focuses on control of bone marrow and systemic (whole-body) disease as well as prevention of cancer at other sites, particularly the central nervous system (CNS). In general, ALL treatment is divided into several phases:  
 
'''Induction chemotherapy''' to bring about remission - that is, leukemic cells are no longer found in bone marrow samples. For adult ALL, standard induction plans include prednisone, vincristine, and an anthracycline drug; other drug plans may include L-asparaginase or cyclophosphamide. For children with low-risk ALL, standard therapy usually consists of three drugs (prednisone, L-asparaginase, and vincristine) for the first month of treatment. High-risk children may receive these drugs plus an anthracycline such as daunorubicin.
 
'''Consolidation therapy''' (1-3 months in adults; 4-8 months in children) to eliminate any leukemia cells that are still "hiding" within the body. A combination of chemotherapeutic drugs is used to keep the remaining leukemia cells from developing resistance. Patients with low- to average-risk ALL receive therapy with antimetabolite drugs such as methotrexate and 6-mercaptopurine (6-MP). High-risk patients receive higher drug doses plus treatment with extra chemotherapeutic agents.
 
'''CNS prophylaxis''' (preventive therapy) to stop the cancer from spreading to the brain and nervous system. Standard prophylaxis may consist of:
# Cranial (head) irradiation plus spinal tap or intrathecal (IT) delivery (into the space around the spinal cord and brain) of the drug methotrexate.
# High-dose systemic and IT methotrexate, without cranial irradiation
# IT chemotherapy.
Only children with T-cell leukemia, a high white blood cell count, or leukemia cells in the cerebrospinal fluid (CSF) need to receive cranial irradiation as well as IT therapy.
 
'''Maintenance treatments''' with chemotherapeutic drugs (e.g., prednisone + vincristine + cyclophosphamide + doxorubicin; methotrexate + 6-MP) to prevent disease recurrence once remission has been achieved. Maintenance therapy usually involves drug doses that are lower than those administered during the induction phase. In children, an intensive 6-month treatment program is needed after induction, followed by 2 years of maintenance chemotherapy.
 
'''Follow-up therapy''' for ALL patients usually consists of:  
* supportive care, such as intravenous nutrition and treatment with oral antibiotics (e.g., ofloxacin, rifampin), especially in patients with prolonged granulocytopenia; that is, too few mature granulocytes (neutrophils), the bacteria-destroying white blood cells that contain small particles, or granules (< 100 granulocytes per cubic millimeter for 2 weeks)
* transfusions with red blood cells and platelets
 
A laboratory test known as [[polymerase chain reaction]] (PCR) is advisable for ALL patients, since it may help to identify specific genetic abnormalities. Such abnormalities have a large impact upon prognosis and, consequently, treatment plans. PCR testing is especially important for patients whose disease is B-cell in type. B-cell ALL usually is not cured by standard ALL therapy. Instead, higher response rates are achieved with the aggressive, cyclophosphamide-based regimens that are used for non-Hodgkin's lymphoma.
 
Among ALL patients, 3-5% children and 25-50% of adults are positive for the Philadelphia chromosome (Ph1). Because these patients have a worse prognosis than other individuals with ALL, many oncologists recommend allogeneic bone marrow transplantation (alloBMT), since remission may be brief following conventional ALL chemotherapy.
 
People who receive bone marrow transplantation will require protective isolation in the hospital, including filtered air, sterile food, and sterilization of the microorganisms in the gut, until their total white blood cell (WBC) count is above 500.
 
Recurrent ALL patients usually do not benefit from additional chemotherapy alone. If possible, they should receive re-induction chemotherapy, followed by allogeneic bone marrow transplant (alloBMT).
 
Alternatively, patients with recurrent ALL may benefit from participation in new clinical trials of alloBMT, immune system agents, and chemotherapeutic agents, or low-dose radiotherapy, if the cancer recurs throughout the body or CNS.
 
===Chronic Lymphocytic Leukemia (CLL)===
CLL is probably incurable by present treatments. But, fortunately, a large group of CLL patients do not require therapy. Studies suggest that people with Stage A CLL (that is, individuals who have fewer than three areas of enlarged lymphoid tissue) do not benefit from early treatment. They may, in fact, suffer drawbacks because of it. Therefore, most oncologists base CLL treatment upon both the stage and symptoms of the patient.
 
For example, in older patients (60+ years) who have low-risk early stage disease (Rai Stage 0) a conservative "watch and wait" approach may be taken.
 
By contrast, older individuals with CLL-related complications or more advanced disease (Rai Stage III or IV) may benefit from chemotherapy and treatment with a corticosteroid (e.g., prednisone, prednisolone).
 
Corticosteroids are first-line agents for people in whom the immune systems has been altered by CLL. CLL may cause autoimmune syndromes in which the patient's immune system attacks and destroys his or her own blood cells. When the red blood cells are affected, the condition is known as immunohemolytic anemia, characterized by decreased numbers of red blood cells, which may cause fatigue, dizziness, and shortness of breath. When the blood platelets are affected, it is called immune-mediated thrombocytopenia, in which a decreased numbers of platelets may lead to bleeding.
 
For younger patients who are experiencing symptoms, the physician may consider early chemotherapy, plus allogeneic or autologous bone marrow transplantation (alloBMT; autoBMT).
 
In general, the indications for treatment are:  
 
* falling hemoglobin or platelet count
* progression to a later stage of disease
* painful, disease-related overgrowth of lymph nodes or spleen
* lymphocyte doubling time (an indicator of lymphocyte reproduction) of fewer than 12 months
 
====Transformation of CLL to high-grade disease or aggressive non-Hodgkin's lymphoma====
If the patient experiences blood flow problems caused by high numbers of leukemia cells in the circulation, the physician may recommend leukapheresis, also known as apheresis, to separate out white blood cells, prior to chemotherapy.
Symptoms that are related to enlargement of the lymph nodes in one area or an overgrown spleen may be treated by localized, low-dose radiotherapy, or surgical management by splenectomy (removal of the spleen). But if leukemia has invaded the lymph nodes at many different sites, total body irradiation (TBI) may be needed.
 
====Chemotherapy for CLL====
The chemotherapeutic plans that are used most often for CLL are:  
 
* combination chemotherapy with chlorambucil (Leukeran®) or cyclophosphamide (Cytoxan®) plus a corticosteroid drug such as prednisone, or
* single-agent treatments with nucleoside drugs such as fludarabine, pentostatin, or cladribine (2-chlorodeoxyadenisine; 2-CDA). However, such drugs usually are reserved for cases in which CLL is resistant (unresponsive to treatment) or returns after chemotherapy with chlorambucil or cyclophosphamide.
 
People with intermediate (Rai Stage I and II) or advanced (Rai Stage III or IV) disease may be helped by participation in a clinical trial. At the present time, clinical trials are being conducted using immunologic compounds (e.g., interferons, monoclonal antibodies) as well as new chemotherapeutic agents (e.g., bryostatin, dolastatin 10, and PSC 83 - a cyclosporine drug given with chemotherapy to overcome drug resistance).
 
===Hairy Cell Leukemia (HCL)===
[[Hairy cell leukemia]] is an incurable, indolent blood disorder in which mutated, partly matured B cells accumulate in the bone marrow.  Its name is derived from the shape of the cells, which look like they are covered with short, fine, hair-shaped projections.  Unlike any other leukemia, HCL is characterized by ''low'' white blood cell counts.
 
Patients with hairy cell leukemia who are symptom-free typically do not receive immediate treatment. They engage in "watchful waiting" with routine bloodwork and exams every three to six months to monitor disease progression and identify any new symptoms.
 
Treatment is generally considered necessary when the patient shows signs and symptoms such as low blood cell counts (e.g., infection-fighting neutrophil count below 1.0 K/ul), frequent infections, unexplained bruises, anemia, or fatigue that is significant enough to disrupt the patient's everyday life.  
 
Patients who need treatment, which includes most newly diagnosed HCL cases, usually receive either [[cladribine]] or [[pentostatin]], which are both in a class of chemotherapeutic drugs known as [[purine]] analogs or [[nucleoside]]s. In most cases, one round of treatment will produce a prolonged remission.
 
Other treatments include [[rituximab]] infusion or self-injection with [[Interferon-alpha]].  In limited cases, the patient may benefit from [[splenectomy]] (removal of the spleen). These treatments are not typically given as the first treatment for a new patient because their success rates are lower than cladribine or pentostatin.
 
In the short term, especially when neutrophil counts are low, an immune system hormone called granulocyte colony-stimulating factor may be taken to increase white blood cell counts.  This is believed to help prevent or treat an infection.  Many patients also take [[antibiotics]] until their white blood cell counts have recovered to normal levels.


==References==
==References==
{{Reflist|2}}
{{Reflist|2}}


== Research ==
[[Category:Medicine]]
 
Significant research into the causes, diagnosis, treatment, and prognosis of leukemia is being done.  Hundreds of [[clinical trials]] are being planned or conducted at any given time.  Studies may focus on effective means of treatment, better ways of treating the disease, improving the quality of life for patients, or appropriate care in remission or after cure.
 
==External links==
'''Images of leukemias'''
*[http://www.healthsystem.virginia.edu/internet/hematology/HessIDB/stages-of-acute-leukemia.cfm UVA Healthsystem] -- Images of acute leukemias by stage
 
'''Research organizations'''
*[http://www.tacl.us/ The Therapeutic Advances in Childhood Leukemia (TACL) Consortium] -- US research group
*[http://atlasgeneticsoncology.org/Educ/Hempat_e.html "Chromosomes, Leukaemias, Solid Tumors, Hereditary Cancers"] in AtlasGeneticsOncology.
 
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{{Hematological malignancy histology}}
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Latest revision as of 22:30, 29 July 2020

Leukemia Microchapters

Home

Patient Information

Overview

Classification

AML
CML
ALL
CLL

Differentiating Leukemia from other Diseases

Epidemiology and Demographics

Prognosis

For patient information click here

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Seyedmahdi Pahlavani, M.D. [2], Usama Talib, BSc, MD [3], Sadaf Sharfaei M.D.[4]; Grammar Reviewer: Natalie Harpenau, B.S.[5]

Synonyms and keywords: Leukaemia

Overview

Leukemia (Greek leukos, “white”; haima, “blood”) can be defined as a group of hematopoietic stem cell malignancies due to genetic abnormalities that may lead to clonal proliferation of these cells. These group of diseases are classified based on the type of hematopoietic stem cell involved and the duration of the disease. The leukemias are the most common malignancies among children younger than 15 years. Among them, Acute Lymphoblastic Leukemia (ALL) is the most common leukemia and accounts for 77% of childhood leukemia. However, Chronic Lymphocytic Leukemia (CLL) is the most common form of leukemia in adults, and it accounts for 30% of all leukemias in the United States. The increased rate of proliferation and decreased rate of apoptosis in this progeny of cells may compromise normal bone marrow function and ultimately result in marrow failure. Clinical manifestations, diagnosis, laboratory findings, and therapy are different according to the type of malignancy.

Classification

Leukemia may be classified as follows:

 
 
 
 
 
 
 
 
Leukemia
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
Lymphoid progeny
 
 
 
 
 
 
 
Myeloid progeny
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
Acute lymphoblastic leukemia (ALL)
 
 
 
Chronic lymphocytic leukemia (CLL)
 
Acute Myeloid Leukemia (AML)
 
 
 
Chronic myeloid leukemia (CML)




Differentiating Leukemia from other Diseases

Leukemia must be differentiated from various diseases that cause weight loss, night sweats, hepatosplenomegaly, and palpable lymph nodes, such as hairy cell leukemia, prolymphocytic leukemia, follicular lymphoma, and mantle cell lymphoma. Based on the expression of cell surface markers, the table below differentiates different types of leukemia from other diseases that cause similar clinical presentations:[1]

Disease Etiology Clinical Manifestation Laboratory Findings Gold standard diagnosis Associated findings
Demography History Symptoms Signs Lab Histopathology
Acute myelogenous leukemia[2][3]
  • Clonal proliferation of malignant myeloid blast cells in the bone marrow
  • Genetic abnormalities t(8;21), inv(16), and t(15;17)
  • The most common leukemia in adults
  • Median age of 63 years old
Acute lymphoblastic leukemia[4][5]
  • Arrest of lymphoblasts
  • Chromosomal translocations: t(9;22) , t(12;21), t(5;14), t(1;19)
  • The most common cancer in children
  • Peak 2-5 years of age
  • Boys > girls
  • History of cancer
  • History of drug exposure
  • CNS involvement
Chronic myelogenous leukemia[6][7]
  • Median age 50 years old
Disease Etiology Demography History Symptoms Signs Lab Histopathology Gold standard diagnosis Associated findings
Chronic lymphocytic leukemia[8]
  • The most common leukemia in adults in western countries
  • M > F
  • Median age 70 years old
Hairy cell leukemia[9][10]
  • Accumulation of small mature B cell lymphoid cells with abundant cytoplasm and "hairy" projections
  • BRAF mutation
  • Uncommon
  • Median age 50 to 55 years old
  • M >> F
  • More common in Caucasians than Blacks
Large granular lymphocytic leukemia[11][12]
  • Rare
  • Median age 60 years
  • M = F
  • Mostly asymptomatic
  • Large lymphocytes with a condensed round or oval nucleus, abundant pale basophilic cytoplasm, and small azurophilic granules
  • Biopsy and flow cytometry + T-cell receptor gene rearrangement studies
  • Recurrent bacterial infection
Chronic neutrophilic leukemia[13]
  • Mature granulocytic proliferation in the blood and bone marrow
  • Point mutations in the CSF3R gene
  • Very rare
  • M = F
  • Toxic granulation in the neutrophils
  • Nuclear hypersegmentation
  • Increased myeloid:erythroid ratio > 20:1
  • WHO diagnostic criteria include leukocytosis of ≥ 25 x 109/L
  • More than 80% neutrophils,
  • Less than 10% circulating neutrophil precursors with blasts
  • Poor prognosis
  • Absence of the Philadelphia chromosome or a BCR/ABL fusion gene
Disease Etiology Demography History Symptoms Signs Lab Histopathology Gold standard diagnosis Associated findings
Chronic eosinophilic leukemia
  • There is no known cause for chronic eosinophilic leukemia.
  • It hasn't been linked to a specific chromosome or genetic abnormality.
  • Unknown
  • Unknown
Chronic monocytic leukemia
Prolymphocytic leukemia (PLL)
T-cell large granular lymphocytic leukemia (TLGL)
Disease Etiology Demography History Symptoms Signs Lab Histopathology Gold standard diagnosis Associated findings
Aggressive NK-cell leukemia (ANKL)
Adult T-cell leukemia/lymphoma (ATLL)[14][15][16][17][18][19]
  • Adult T-cell leukemia is caused by an infection with HTLV.
  • Common genetic mutations involved in the development of adult T-cell leukemia can be found here.
  • The incidence of adult T-cell leukemia increases with age, and the median age at diagnosis is 57 years.
  • Males are more commonly affected with adult T-cell leukemia than females.
  • The male to female ratio is approximately 1.4 to 1.
Sezary syndrome[20][21][22][23][24][25][26][27]
  • The cause of Sezary syndrome has not been identified.
  • Sezary syndrome might have one or more of the chromosomal abnormalities, such as the loss or gain of genetic.
  • The prevalence of Sezary syndrome is exact unknown.
  • The median age at diagnosis of Sézary syndrome is 60 years of age.
  • Sezary syndrome is more commonly observed among elderly patients.
  • Males are more commonly affected with Sezary syndrome than females(2:1).
Myelodysplastic syndrome Biopsy
  • Leukemia transformation
  • Acquired pseudo-Pelger-Huët anomaly
  • Infection
Myeloproliferative disorders
Leukemoid reaction

Epidemiology and Demographics

Prevalence

  • In the United States, the age-adjusted prevalence of leukemia is 75.3 per 100,000 in 2011.[29]

Incidence

  • The delay-adjusted incidence of leukemia in 2011 was estimated as 15.48 per 100,000 individuals in the United States.
  • In 2011, the age-adjusted incidence of leukemia was 13.66 per 100,000 individuals in the United States.

Age

  • The overall age-adjusted incidence of leukemia in the United States between 2007 and 2011 was 13 per 100,000 occurrences. The age-adjusted incidence of leukemia by age category is:
    • Under 65 years: 6.5 per 100,000
    • 65 and over: 57.9 per 100,000
  • Shown below is a table depicting the overall age-adjusted incidence of leukemia per 100,000 individuals by age in the United States between 2007 and 2011 for the different types of leukemia.
Acute lymphoblastic leukemia Chronic lymphocytic leukemia Acute myeloid leukemia Chronic myeloid leukemia
All ages 1.7 4.4 3.8 1.7
<65 1.7 1.4 1.8 0.9
≥65 1.6 25.2 17.5 6.8

Gender

  • In the United States, the age-adjusted prevalence of leukemia by gender in 2011 was:
    • In males: 92.7 per 100,000
    • In females: 60.7 per 100,000
  • In the United States, the delay-adjusted incidence of leukemia by gender in 2011 was:
    • In males: 19.93 per 100,000 persons
    • In females: 11.89 per 100,000 persons
  • In the United States, the age-adjusted incidence of leukemia by gender on 2011 was:
    • In males: 17.58 per 100,000 persons
    • In females: 10.49 per 100,000 persons
  • Shown below is an image depicting the delay-adjusted incidence and observed incidence of leukemia by gender and race in the United States between 1975 and 2011. These graphs were gathered from SEER: The Surveillance, Epidemiology, and End Results Program of the National Cancer Institute.
These graphs are adapted from SEER: The Surveillance, Epidemiology, and End Results Program of the National Cancer Institute. - Delay-adjusted incidence and observed incidence of leukemia by gender and race in the United States between 1975 and 2011

Race

  • Shown below is a table depicting the age-adjusted prevalence of leukemia by race in 2011 in the United States.
All Races White Black Asian/Pacific Islander Hispanic
Age-adjusted prevalence 75.3 per 100,000 83.5 per 100,000 45.9 per 100,000 41.2 per 100,000 57.1 per 100,000
  • Shown below is an image depicting the incidence of leukemia by race in the United States between 1975 and 2011.

Incidence of leukemia by race in the United States between 1975 and 2011

API: Asian/Pacific Islander; AI/AN: American Indian/ Alaska Native

Prognosis

5-Year Survival

  • Between 2004 and 2010, the 5-year relative survival of patients with leukemia was 60.3%.
  • When stratified by age, the 5-year relative survival of patients with leukemia was 68.5% (44.1% for patients <65 and ≥ 65 years of age respectively).
  • Shown below is a table depicting the 5-year relative survival of patients by the type of leukemia in the United States between 2004 and 2010.
Acute lymphoblastic leukemia Chronic lymphocytic leukemia Acute myeloid leukemia Chronic myeloid leukemia
5-year survival 70% 83.5% 25.4% 59.9%

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