Hairy cell leukemia overview: Difference between revisions
No edit summary |
|||
| Line 4: | Line 4: | ||
==Overview== | ==Overview== | ||
Hairy cell leukemia arises from [[B cell]]s, that are normally involved in the process of human [[immunoglobulin]]s production.<ref name="m">Magrath I. The Lymphoid Neoplasms 3ed. CRC Press; 2010.</ref> However, the exact B cell maturation stage involved in the development of hairy cell leukemia is still unclear.<ref name="found">What is Hairy Cell Leukemia? Hairy Cell Leukemia Foundation (2015) https://www.hairycellleukemia.org/about-hcl/what-is-hairy-cell-leukemia/ Accessed on October, 19 2015</ref> The most common gene involved in the pathogenesis of hairy cell leukemia is BRAF V600E | Hairy cell leukemia arises from [[B cell]]s, that are normally involved in the process of human [[immunoglobulin]]s production.<ref name="m">Magrath I. The Lymphoid Neoplasms 3ed. CRC Press; 2010.</ref> However, the exact B cell maturation stage involved in the development of hairy cell leukemia is still unclear.<ref name="found">What is Hairy Cell Leukemia? Hairy Cell Leukemia Foundation (2015) https://www.hairycellleukemia.org/about-hcl/what-is-hairy-cell-leukemia/ Accessed on October, 19 2015</ref> The most common gene involved in the pathogenesis of hairy cell leukemia is BRAF V600E mutation.<ref name="wiki"> Hairy cell leukemia. Wikipedia (2015) https://en.wikipedia.org/wiki/Hairy_cell_leukemia#Pathophysiology Accessed on Ocotber, 19 2015</ref> On microscopic histopathological analysis, characteristic findings of hairy cell leukemia include a small cells with "Fried egg"-like appearance, well-demarcated fuzzy borders, and a clear cytoplasm.<ref name="patho">Hairy cell leukemia. Libre Pathology (2015) http://librepathology.org/wiki/index.php/Small_cell_lymphomas#Hairy_cell_leukemia Accessed on October, 8 2015</ref> Hairy cell leukemia is found to approximately comprise 2% of all leukemias in the United States.<ref name="wiki">Hairy Cell Leukemia. Wikipedia (2015) https://en.wikipedia.org/wiki/Hairy_cell_leukemia#Pathophysiology Accesed on October, 20 2015</ref> The incidence of hairy cell leukemia is approximately 3.2 per 1,000,000 individuals in the United States. The incidence of hairy cell leukemia increases with age; the median age at diagnosis is 58 years. Males are more commonly affected with hairy cell leukemia than females. The male to female ratio is approximately 4 to 1.<ref name="found"> Epidemiology. HAIRY CELL LEUKEMIA FOUNDATION. (2015) https://www.hairycellleukemia.org/professionals/epidemiology/ Accessed on October, 21 2015</ref> Studies have proposed a number of risk factors for the development of hairy cell leukmeia such as certain chemicals exposure, radiation exposure, and sawdust exposure.<ref name="may">Hairy cell leukemia. MAYO CLINIC (2015) http://www.mayoclinic.org/diseases-conditions/hairy-cell-leukemia/basics/risk-factors/con-20026432 Accessed on October, 20 2015</ref> Symptoms of hairy cell leukemia include [[fever]], night sweats, and [[weight loss]].<ref name="wiki">Hairy cell leukemia. Wikipedia (2015) https://en.wikipedia.org/wiki/Hairy_cell_leukemia#Signs_and_symptoms Accessed on October, 28 2015</ref><ref name="gov">Hairy cell leukemia. National Cancer Institute (2015) http://www.cancer.gov/types/leukemia/hp/hairy-cell-treatment-pdq Accessed on October, 28 2015</ref> Physical examination of patients with hairy cell leukemia is usually remarkable for skin [[pallor]], [[petechiae]], and [[splenomegaly]].<ref name="found"> Epidemiology. HAIRY CELL LEUKEMIA FOUNDATION. (2015) https://www.hairycellleukemia.org/professionals/epidemiology/ Accessed on October, 28 2015</ref> Laboratory findings consistent with the diagnosis of hairy cell leukemia include abnormal complete blood count, immunohistochemistry, and flow cytometry. The presence of hairy cells with thread-like [[cytoplasmic]] extensions on blood smear is a key diagnostic feature among patients with hairy cell leukemia.<ref name="pub">{{cite journal |vauthors=Somasundaram V, Purohit A, Aggarwal M, Manivannan P, Mishra P, Seth T, Tyagi S, Mahapatra M, Pati HP, Saxena R |title=Hairy cell leukemia: A decade long experience of North Indian Hematology Center |journal=Indian J Med Paediatr Oncol |volume=35 |issue=4 |pages=271–5 |year=2014 |pmid=25538404 |pmc=4264273 |doi=10.4103/0971-5851.144988 |url=}}</ref><ref name="patho">Small cell lymphoma. Libre Pathology (2015) http://librepathology.org/wiki/index.php/Small_cell_lymphomas#Hairy_cell_leukemia Accessed on October, 8 2015</ref> There is no '''curative''' treatment for hairy cell leukemia. The mainstay of therapy for hairy cell leukemia patients is chemotherapy. Pharmacological agents used for the treatment of hairy cell leukemia patients include [[cladribine]], [[pentostatin]], [[rituximab]], and <br>[[vemurafenib]].<ref name="wiki"> Hairy cell leukemia. Wikipedia (2015) https://en.wikipedia.org/wiki/Hairy_cell_leukemia#Pathophysiology Accessed on Ocotber, 25 2015</ref><ref name="NCCN">NCCN Guidelines Version 2.2015 CLL/SLL. National Comprehensive Cancer Network. (2015) http://www.nccn.org/professionals/physician_gls/PDF/nhl.pdf Accessed on October, 25 2015</ref><ref name="pub4">National Cancer Institute: PDQ® Hairy Cell Leukemia Treatment. Bethesda, MD: National Cancer Institute. http://www.cancer.gov/types/leukemia/hp/hairy-cell-treatment-pdq. Accessed October, 25 2015</ref><ref name="pub7">{{cite journal |vauthors=Cornet E, Damaj G, Troussard X |title=New insights in the management of patients with hairy cell leukemia |journal=Curr Opin Oncol |volume=27 |issue=5 |pages=371–6 |year=2015 |pmid=26154707 |doi=10.1097/CCO.0000000000000214 |url=}}</ref> | ||
==Historical Perspective== | ==Historical Perspective== | ||
The term hairy cell leukemia was first used to describe the malignancy by Dr. R. Schrek and Dr. W. J. Donnelly, in 1966.<ref name="pmid18283787">{{cite journal| author=Fanta PT, Saven A| title=Hairy cell leukemia. | journal=Cancer Treat Res | year= 2008 | volume= 142 | issue= | pages= 193-209 | pmid=18283787 | doi= | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=18283787 }} </ref> | The term hairy cell leukemia was first used to describe the malignancy by Dr. R. Schrek and Dr. W. J. Donnelly, in 1966.<ref name="pmid18283787">{{cite journal| author=Fanta PT, Saven A| title=Hairy cell leukemia. | journal=Cancer Treat Res | year= 2008 | volume= 142 | issue= | pages= 193-209 | pmid=18283787 | doi= | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=18283787 }} </ref> | ||
Revision as of 18:03, 30 October 2015
|
Hairy cell leukemia Microchapters |
|
Diagnosis |
|---|
|
Treatment |
|
Case Studies |
|
Hairy cell leukemia overview On the Web |
|
American Roentgen Ray Society Images of Hairy cell leukemia overview |
|
Risk calculators and risk factors for Hairy cell leukemia overview |
Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1] Associate Editor(s)-in-Chief: Haytham Allaham, M.D. [2]
Overview
Hairy cell leukemia arises from B cells, that are normally involved in the process of human immunoglobulins production.[1] However, the exact B cell maturation stage involved in the development of hairy cell leukemia is still unclear.[2] The most common gene involved in the pathogenesis of hairy cell leukemia is BRAF V600E mutation.[3] On microscopic histopathological analysis, characteristic findings of hairy cell leukemia include a small cells with "Fried egg"-like appearance, well-demarcated fuzzy borders, and a clear cytoplasm.[4] Hairy cell leukemia is found to approximately comprise 2% of all leukemias in the United States.[3] The incidence of hairy cell leukemia is approximately 3.2 per 1,000,000 individuals in the United States. The incidence of hairy cell leukemia increases with age; the median age at diagnosis is 58 years. Males are more commonly affected with hairy cell leukemia than females. The male to female ratio is approximately 4 to 1.[2] Studies have proposed a number of risk factors for the development of hairy cell leukmeia such as certain chemicals exposure, radiation exposure, and sawdust exposure.[5] Symptoms of hairy cell leukemia include fever, night sweats, and weight loss.[3][6] Physical examination of patients with hairy cell leukemia is usually remarkable for skin pallor, petechiae, and splenomegaly.[2] Laboratory findings consistent with the diagnosis of hairy cell leukemia include abnormal complete blood count, immunohistochemistry, and flow cytometry. The presence of hairy cells with thread-like cytoplasmic extensions on blood smear is a key diagnostic feature among patients with hairy cell leukemia.[7][4] There is no curative treatment for hairy cell leukemia. The mainstay of therapy for hairy cell leukemia patients is chemotherapy. Pharmacological agents used for the treatment of hairy cell leukemia patients include cladribine, pentostatin, rituximab, and
vemurafenib.[3][8][9][10]
Historical Perspective
The term hairy cell leukemia was first used to describe the malignancy by Dr. R. Schrek and Dr. W. J. Donnelly, in 1966.[11]
Classification
Hairy cell leukemia may be classified into two subtypes: Hairy cell leukemia (classic) and hairy cell leukemia variant.[3]
Pathogenesis
Hairy cell leukemia arises from B cells, that are normally involved in the process of human immunoglobulins production.[1] However, the exact B cell maturation stage involved in the development of hairy cell leukemia is still unclear.[2] The most common gene involved in the pathogenesis of hairy cell leukemia is BRAF V600E mutations.[3] On microscopic histopathological analysis, characteristic findings of hairy cell leukemia include a small cells with "Fried egg"-like appearance, well-demarcated fuzzy borders, and a clear cytoplasm.[4]
Causes
There are no established direct causes for hairy cell leukemia. Common genetic mutations involved in the development of hairy cell leukemia can be found here.[3][12]
Epidemiology and Demographics
Hairy cell leukemia is found to approximately comprise 2% of all leukemias in the United States.[3] The incidence of hairy cell leukemia is approximately 3.2 per 1,000,000 individuals in the United States. The incidence of hairy cell leukemia increases with age; the median age at diagnosis is 58 years. Males are more commonly affected with hairy cell leukemia than females. The male to female ratio is approximately 4 to 1.[2]
Risk Factors
Studies have proposed a number of risk factors for the development of hairy cell leukmeia such as certain chemicals exposure, radiation exposure, and sawdust exposure.[5]
Screening
According to the the U.S. Preventive Service Task Force (USPSTF), there is insufficient evidence to recommend routine screening for hairy cell leukemia.[13]
Natural History
Most patients with hairy cell leukemia are asymptomatic at time of diagnosis. If left untreated, most of the patients with hairy cell leukemia will gradually develop anemia, recurrent infections, and recurrent bleeding. Common complications of hairy cell leukemia are mainly due to bone marrow failure.[3][6][2]
Diagnosis
Staging
There is no established system for the staging of hairy cell leukemia.
History and Symptoms
Symptoms of hairy cell leukemia include fever, night sweats, and weight loss.[3][6]
Physical Examination
Physical examination of patients with hairy cell leukemia is usually remarkable for skin pallor, petechiae, and splenomegaly.[2]
Laboratory Findings
Laboratory findings consistent with the diagnosis of hairy cell leukemia include abnormal complete blood count, immunohistochemistry, and flow cytometry. The presence of hairy cells with thread-like cytoplasmic extensions on blood smear is a key diagnostic feature among patients with hairy cell leukemia.[7][4]
CT Scan
Abdominal CT scan may be helpful in the diagnosis of hairy cell leukemia. Findings on CT scan suggestive of hairy cell leukemia include splenomegaly.[14]
MRI
MRI of the femur may be helpful in the diagnosis of hairy cell leukemia. Diffuse hyperintense lesions on a T2-weighted MRI image is suggestive of bone marrow infiltration by malignant leukemic cells.[15][16][17]
Medical Therapy
There is no curative treatment for hairy cell leukemia. The mainstay of therapy for hairy cell leukemia patients is chemotherapy. Pharmacological agents used for the treatment of hairy cell leukemia patients include cladribine, pentostatin, rituximab, and vemurafenib.[8][3][9][10]
Surgery
The predominant therapy for hairy cell leukemia is chemotherapy. Surgical management, such as splenectomy, may be required in certain cases.[3]
References
- ↑ 1.0 1.1 Magrath I. The Lymphoid Neoplasms 3ed. CRC Press; 2010.
- ↑ 2.0 2.1 2.2 2.3 2.4 2.5 2.6 What is Hairy Cell Leukemia? Hairy Cell Leukemia Foundation (2015) https://www.hairycellleukemia.org/about-hcl/what-is-hairy-cell-leukemia/ Accessed on October, 19 2015
- ↑ 3.00 3.01 3.02 3.03 3.04 3.05 3.06 3.07 3.08 3.09 3.10 3.11 Hairy cell leukemia. Wikipedia (2015) https://en.wikipedia.org/wiki/Hairy_cell_leukemia#Pathophysiology Accessed on Ocotber, 19 2015
- ↑ 4.0 4.1 4.2 4.3 Hairy cell leukemia. Libre Pathology (2015) http://librepathology.org/wiki/index.php/Small_cell_lymphomas#Hairy_cell_leukemia Accessed on October, 8 2015
- ↑ 5.0 5.1 Hairy cell leukemia. MAYO CLINIC (2015) http://www.mayoclinic.org/diseases-conditions/hairy-cell-leukemia/basics/risk-factors/con-20026432 Accessed on October, 20 2015
- ↑ 6.0 6.1 6.2 Hairy cell leukemia. National Cancer Institute (2015) http://www.cancer.gov/types/leukemia/hp/hairy-cell-treatment-pdq Accessed on October, 28 2015
- ↑ 7.0 7.1 Somasundaram V, Purohit A, Aggarwal M, Manivannan P, Mishra P, Seth T, Tyagi S, Mahapatra M, Pati HP, Saxena R (2014). "Hairy cell leukemia: A decade long experience of North Indian Hematology Center". Indian J Med Paediatr Oncol. 35 (4): 271–5. doi:10.4103/0971-5851.144988. PMC 4264273. PMID 25538404.
- ↑ 8.0 8.1 NCCN Guidelines Version 2.2015 CLL/SLL. National Comprehensive Cancer Network. (2015) http://www.nccn.org/professionals/physician_gls/PDF/nhl.pdf Accessed on October, 25 2015
- ↑ 9.0 9.1 National Cancer Institute: PDQ® Hairy Cell Leukemia Treatment. Bethesda, MD: National Cancer Institute. http://www.cancer.gov/types/leukemia/hp/hairy-cell-treatment-pdq. Accessed October, 25 2015
- ↑ 10.0 10.1 Cornet E, Damaj G, Troussard X (2015). "New insights in the management of patients with hairy cell leukemia". Curr Opin Oncol. 27 (5): 371–6. doi:10.1097/CCO.0000000000000214. PMID 26154707.
- ↑ Fanta PT, Saven A (2008). "Hairy cell leukemia". Cancer Treat Res. 142: 193–209. PMID 18283787.
- ↑ Tiacci E, Liso A, Piris M, Falini B (2006). "Evolving concepts in the pathogenesis of hairy-cell leukaemia". Nat Rev Cancer. 6 (6): 437–48. doi:10.1038/nrc1888. PMID 16723990.
- ↑ Recommendations. US preventive services task force (2015) http://www.uspreventiveservicestaskforce.org/BrowseRec/Search?s=Hairy+cell+leukemia+ Accessed on October, 12 2015
- ↑ Diseases and conditions Hairy cell leukemia. MAYO CLINIC (2015) http://www.mayoclinic.org/diseases-conditions/hairy-cell-leukemia/basics/tests-diagnosis/con-20026432 Accessed on October, 28 2015
- ↑ Silingardi V, Davolio-Marani S, Federico M, Piccinini L, Frassoldati A, Sarti M, Burani A, Canossi G (1989). "Bone marrow infiltration in hairy cell leukemia after interferon therapy detected by magnetic resonance imaging". Eur J Cancer Clin Oncol. 25 (2): 209–13. PMID 2702977.
- ↑ Thompson JA, Shields AF, Porter BA, Olson DO, Rubin E, Kidd P, Fefer A (1987). "Magnetic resonance imaging of bone marrow in hairy cell leukemia: correlation with clinical response to alpha-interferon". Leukemia. 1 (4): 315–6. PMID 3669752.
- ↑ Gray MT, Rutherford MN, Bonin DM, Patterson B, Lopez PG (2013). "Hairy-cell leukemia presenting as lytic bone lesions". J. Clin. Oncol. 31 (25): e410–2. doi:10.1200/JCO.2012.47.5301. PMID 23797001.