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==Overview== | ==Overview== | ||
'''Hairy cell leukemia''' is a rare neoplasm of small B-lymphocytes with "hairy" projections in [[bone marrow]], [[spleen]], and [[peripheral blood]]. Most patients are middle-aged to elderly adults and present with [[splenomegaly]] and [[pancytopenia]]. (WHO, 2001) | '''Hairy cell leukemia''' is a rare neoplasm of small B-lymphocytes with "hairy" projections in [[bone marrow]], [[spleen]], and [[peripheral blood]]. Most patients are middle-aged to elderly adults and present with [[splenomegaly]] and [[pancytopenia]]. (WHO, 2001) | ||
==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> | |||
==Classification== | |||
Hairy cell leukemia may be classified into two subtypes: Hairy cell leukemia (classic) and hairy cell leukemia variant.<ref name="wiki">Hairy cell leukemia. Wikipedia (2015) https://en.wikipedia.org/wiki/Hairy_cell_leukemia#Classification Accessed on October, 17 2015</ref> | |||
==Pathogenesis== | |||
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 mutations.<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> | |||
==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 [[Hairy cell leukemia pathophysiology|'''here''']].<ref name="wiki"> Hairy cell leukemia. Wikipedia (2015) https://en.wikipedia.org/wiki/Hairy_cell_leukemia#Pathophysiology Accessed on Ocotber, 19 2015</ref><ref name="pmid16723990">{{cite journal| author=Tiacci E, Liso A, Piris M, Falini B| title=Evolving concepts in the pathogenesis of hairy-cell leukaemia. | journal=Nat Rev Cancer | year= 2006 | volume= 6 | issue= 6 | pages= 437-48 | pmid=16723990 | doi=10.1038/nrc1888 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=16723990 }} </ref> | |||
==Epidemiology and Demographics== | |||
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> | |||
==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.<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> | |||
==Screening== | |||
According to the the U.S. Preventive Service Task Force (USPSTF), there is insufficient evidence to recommend routine screening for hairy cell leukemia.<ref name="US">Recommendations. US preventive services task force (2015) http://www.uspreventiveservicestaskforce.org/BrowseRec/Search?s=Hairy+cell+leukemia+ Accessed on October, 12 2015</ref> | |||
==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]].<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><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> | |||
==References== | ==References== | ||
Revision as of 02:37, 30 October 2015
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]
Overview
Hairy cell leukemia is a rare neoplasm of small B-lymphocytes with "hairy" projections in bone marrow, spleen, and peripheral blood. Most patients are middle-aged to elderly adults and present with splenomegaly and pancytopenia. (WHO, 2001)
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.[1]
Classification
Hairy cell leukemia may be classified into two subtypes: Hairy cell leukemia (classic) and hairy cell leukemia variant.[2]
Pathogenesis
Hairy cell leukemia arises from B cells, that are normally involved in the process of human immunoglobulins production.[3] However, the exact B cell maturation stage involved in the development of hairy cell leukemia is still unclear.[4] The most common gene involved in the pathogenesis of hairy cell leukemia is BRAF V600E mutations.[2] 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.[5]
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.[2][6]
Epidemiology and Demographics
Hairy cell leukemia is found to approximately comprise 2% of all leukemias in the United States.[2] 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.[4]
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.[7]
Screening
According to the the U.S. Preventive Service Task Force (USPSTF), there is insufficient evidence to recommend routine screening for hairy cell leukemia.[8]
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.[2][9][4]
References
- ↑ Fanta PT, Saven A (2008). "Hairy cell leukemia". Cancer Treat Res. 142: 193–209. PMID 18283787.
- ↑ 2.0 2.1 2.2 2.3 2.4 Hairy cell leukemia. Wikipedia (2015) https://en.wikipedia.org/wiki/Hairy_cell_leukemia#Classification Accessed on October, 17 2015
- ↑ Magrath I. The Lymphoid Neoplasms 3ed. CRC Press; 2010.
- ↑ 4.0 4.1 4.2 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
- ↑ Hairy cell leukemia. Libre Pathology (2015) http://librepathology.org/wiki/index.php/Small_cell_lymphomas#Hairy_cell_leukemia Accessed on October, 8 2015
- ↑ 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.
- ↑ 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
- ↑ Recommendations. US preventive services task force (2015) http://www.uspreventiveservicestaskforce.org/BrowseRec/Search?s=Hairy+cell+leukemia+ Accessed on October, 12 2015
- ↑ Hairy cell leukemia. National Cancer Institute (2015) http://www.cancer.gov/types/leukemia/hp/hairy-cell-treatment-pdq Accessed on October, 28 2015