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==Overview== | ==Overview== | ||
==Pathogenesis== | ==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> | |||
* Hairy cell leukemia may also infiltrate the [[spleen]] and [[liver]]. | |||
* Extravascular hemolysis may develop due to splenic sequestration and destruction of circulating red blood cells. | |||
* Hairy cell leukemia '''rarely''' infiltrate the peripheral [[lymph node]]s. | |||
* Bone marrow failure may develop among hairy cell leukemia patients due to: | |||
:* [[Malignant]] cells infiltrateion of the [[bone marrow]] | |||
:* Reticulin fibrosis of the bone marrow | |||
:* Dysregulated cytokine production | |||
* The development of [[bone marrow]] failure interferes with the normal production of [[red blood cell]]s and [[platelet]]s among hairy cell leukemia patients. | |||
* Production of [[cytokine]]s, such as TNF α and IL-2R, provide important stimuli for [[malignant]] [[B cell]]s proliferation in hairy cell leukemia. | |||
* In approximately 40% of hairy cell leukemia cases, malignant cells co-express multiple colonally related IgG, IgA, and IgM isotypes.<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> | |||
==Genetics== | ==Genetics== | ||
Revision as of 13:49, 20 October 2015
Overview
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]
- Hairy cell leukemia may also infiltrate the spleen and liver.
- Extravascular hemolysis may develop due to splenic sequestration and destruction of circulating red blood cells.
- Hairy cell leukemia rarely infiltrate the peripheral lymph nodes.
- Bone marrow failure may develop among hairy cell leukemia patients due to:
- Malignant cells infiltrateion of the bone marrow
- Reticulin fibrosis of the bone marrow
- Dysregulated cytokine production
- The development of bone marrow failure interferes with the normal production of red blood cells and platelets among hairy cell leukemia patients.
- Production of cytokines, such as TNF α and IL-2R, provide important stimuli for malignant B cells proliferation in hairy cell leukemia.
- In approximately 40% of hairy cell leukemia cases, malignant cells co-express multiple colonally related IgG, IgA, and IgM isotypes.[3]
Genetics
- The most common gene involved in the pathogenesis of hairy cell leukemia is BRAF V600E mutations.[4]
- The BRAF V600E mutations is present in all patients with hairy cell leukemia (classic).
- The BRAF V600E mutations is not present in patients with hairy cell leukemia (variant).
- Other genes involved in the pathogenesis of hairy cell leukemia may include:[4]
- Under expression of chromosomes 3p24, 3p21, 3q13.3-q22, 4p16, 11q23, 14q22-q24, 15q21-q22, 15q24-q25, and 17q22-q24.
- Over expression of chromosomes 13q31 and Xq13.3-q21
Associated Conditions
Gross Pathology
Microscopic Pathology
- ↑ Magrath I. The Lymphoid Neoplasms 3ed. CRC Press; 2010.
- ↑ 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
- ↑ 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.
- ↑ 4.0 4.1 Hairy cell leukemia. Wikipedia (2015) https://en.wikipedia.org/wiki/Hairy_cell_leukemia#Pathophysiology Accessed on Ocotber, 19 2015