Chronic myelogenous leukemia natural history: Difference between revisions

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==Overview==
==Overview==
If left untreated, majority of patients with chronic myelogenous leukemia may progress from a chronic phase where differentiation is reasonably well-maintained to blast or acute phase (BP) where differentiation is lost. some complications of chronic myelogenous leukemia include Fatigue, excess bleeding, enlarged spleen, and infection. Prognosis is generally poor, and the 5-year survival rate of patients with chronic myelogenous leukemia is approximately 59.9%.<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><ref name="cancergov">National Cancer Institute. Physician Data Query Database 2015.http://www.cancer.gov/types/leukemia/hp/cml-treatment-pdq#link/_381_toc</ref>targeted therapy with small molecule tyrosine kinase inhibitors (TKIs) dramatically alter the natural history of the disease, improving 10-year overall survival (OS) from 20 to 80–90%. PMID:23090888  PMID:24337217
If left untreated, majority of patients with chronic myelogenous leukemia may progress from a chronic phase where differentiation is reasonably well-maintained to blast or acute phase (BP) where differentiation is lost. the progression to BP occurs at a median of 3–5 years from diagnosis in untreated patients. some complications of chronic myelogenous leukemia include Fatigue, excess bleeding, enlarged spleen, and infection. Prognosis is generally poor, and the 5-year survival rate of patients with chronic myelogenous leukemia is approximately 59.9%.<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><ref name="cancergov">National Cancer Institute. Physician Data Query Database 2015.http://www.cancer.gov/types/leukemia/hp/cml-treatment-pdq#link/_381_toc</ref>targeted therapy with small molecule tyrosine kinase inhibitors (TKIs) dramatically alter the natural history of the disease, improving 10-year overall survival (OS) from 20 to 80–90%. PMID:23090888  PMID:24337217 PMID:25993200  PMID:26434969
 
Complications
 
* Fatigue. If diseased white blood cells crowd out healthy red blood cells, anemia may result. Anemia can make you feel tired and worn down. Treatment for <abbr>CML</abbr> also can cause a drop in red blood cells.
* Excess '''bleeding.''' Blood cells called platelets help control bleeding by plugging small leaks in blood vessels and helping your blood to clot. A shortage of blood platelets (thrombocytopenia) can result in easy bleeding and bruising, including frequent or severe nosebleeds, bleeding from the gums, or tiny red dots caused by bleeding into the skin (petechiae).
* '''Pain.''' <abbr>CML</abbr> can cause bone pain or joint pain as the bone marrow expands when excess white blood cells build up.
* '''Enlarged spleen.''' Some of the extra blood cells produced when you have <abbr>CML</abbr> are stored in the spleen. This can cause the spleen to become swollen or enlarged. The swollen spleen takes up space in your abdomen and makes you feel full even after small meals or causes pain on the left side of your body below your ribs.
* Infection'''.''' White blood cells help the body fight off infection. Although people with <abbr>CML</abbr> have too many white blood cells, these cells are often diseased and don't function properly. As a result, they aren't able to fight infection as well as healthy white cells can. In addition, treatment can cause your white cell count to drop too low (neutropenia), also making you vulnerable to infection.
* '''Death.''' If <abbr>CML</abbr> can't be successfully treated, it ultimately is fatal.
Clinical staging of chronic myeloid leukemia (CML) distinguishes between chronic phase (CP-CML), accelerated phase (AP-CML), and blastic phase (BP-CML), reflecting its natural history in the absence of effective therapy.
 
PMID:
: 25993200
: The disease is classically staged into chronic phase (CP, most patients at presentation), accelerated phase (AP) and blast phase (BP).<sup>11</sup> Many definitions have been used for these stages, but all the data generated from the tyrosine kinase inhibitor (TKI) studies has used the historically standard definition where AP is defined by the presence of one or more of the following: ≥15% blasts in PB/BM, ≥20% basophils in PB, platelets <100,000/µL unrelated to treatment or the development of cytogenetic evolution. Blast phase is defined by the presence of ≥30% blasts in the peripheral blood or bone marrow, the presence of clusters of blasts in marrow or the presence of extramedullary disease with immature cells (i.e., a myeloid sarcoma).<sup>14</sup> Progression to BP occurs at a median of 3–5 years from diagnosis in untreated patients, with or without an intervening identifiable AP. PMID:26434969
;
 
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==Natural History==
==Natural History==

Revision as of 19:23, 14 May 2018

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1] Associate Editor(s)-in-Chief: Mohamad Alkateb, MBBCh [2]

Overview

If left untreated, majority of patients with chronic myelogenous leukemia may progress from a chronic phase where differentiation is reasonably well-maintained to blast or acute phase (BP) where differentiation is lost. the progression to BP occurs at a median of 3–5 years from diagnosis in untreated patients. some complications of chronic myelogenous leukemia include Fatigue, excess bleeding, enlarged spleen, and infection. Prognosis is generally poor, and the 5-year survival rate of patients with chronic myelogenous leukemia is approximately 59.9%.[1][2]targeted therapy with small molecule tyrosine kinase inhibitors (TKIs) dramatically alter the natural history of the disease, improving 10-year overall survival (OS) from 20 to 80–90%. PMID:23090888 PMID:24337217 PMID:25993200 PMID:26434969

Natural History

The symptoms of chronic myelogenous leukemia usually develop in the sixth decade of life, and start with symptoms such as fatigue, malaise, and loss of appetite. Without treatment, the patient will develop symptoms of anemia, which may eventually lead to death.[3]

Complications

Chronic myelogenous leukemia may lead to the following complications:[3][4]

  • Leukemia cutis
  • Leukemic cells enter the skin. The sores or patches can be any size and are usually pink or tan in color.
  • Sores usually appear on the extremities
  • Sweet's syndrome (acute febrile neutrophilic dermatosis)
  • Fever
  • Painful sores that may appear anywhere on the body

Prognosis

Prognosis is generally good. Between 2004 and 2010, the 5-year relative survival of patients with CML was 59.9%. When associated with old age, the 5-year survival rate of patients with chronic myelogenous leukemia was 80.2% and 37.1% for patients <65 and ≥ 65 years of age, respectively.[1]

The prognosis and treatment options depend on the following:[3]

  • The patient’s age
  • Elderly people have a less favorable prognosis
  • The phase of CML
  • Accelerated or blast phase at the time of diagnosis is a less favorable prognostic factor
  • The amount of blasts in the blood or bone marrow
  • A high number of blasts in the blood or bone marrow at diagnosis is a less favorable prognostic factor
  • The size of the spleen at diagnosis
  • Splenomegaly at diagnosis is a less favorable prognostic factor
  • Platelet count
  • A higher number of eosinophils and basophils in the blood samples indicates a less favorable prognosis
  • The Philadelphia chromosome
  • Patients with Philadelphia chromosome (Ph+) at diagnosis have a more favorable prognosis than those who do not have the Philadelphia chromosome (Ph-)
  • Presence of anemia at diagnosis is a less favorable prognostic factor
  • Bone marrow involvement
  • A large number of leukemia cells in the bone marrow at the time of diagnosis is a less favorable prognostic factor
  • Performance status
  • People with a low performance status at the time of diagnosis have a less favorable prognosis
  • Serum lactate dehydrogenase blood level
  • High serum lactate dehydrogenase (LDH) in the blood is a less favorable prognostic factor
  • Response to treatment
  • The treatment is effective if a person has a major cytogenetic response after treatment, which means that less than 30% of a person’s blood cells have the Philadelphia chromosome. A major cytogenetic response occurs in about 50–70% of people considered to have good-risk disease (favorable prognostic factors) and in 20% of people considered to have poor-risk disease (unfavorable prognostic factors).
  • The patient’s general health

References

  1. 1.0 1.1 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.
  2. National Cancer Institute. Physician Data Query Database 2015.http://www.cancer.gov/types/leukemia/hp/cml-treatment-pdq#link/_381_toc
  3. 3.0 3.1 3.2 Canadian Cancer Society.2015.http://www.cancer.ca/en/cancer-information/cancer-type/leukemia-chronic-myelogenous-cml/finding-cancer-early/?region=ab
  4. Medline Plus.2015.https://www.nlm.nih.gov/medlineplus/ency/article/000570.htm


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