Hairy cell leukemia pathophysiology: Difference between revisions

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* On microscopic histopathological analysis, characteristic findings of hairy cell leukemia include:<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>
* On microscopic histopathological analysis, characteristic findings of hairy cell leukemia include:<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>
:* Small cells with "fried egg"-like appearance
:* Small cells with "fried egg"-like appearance
:* Well-demarcated thread-like cytoplasmic extensions
:* Well-demarcated thread-like [[cytoplasmic]] extensions
:* Clear cytoplasm
:* Clear cytoplasm
:* Central round nucleus
:* Central round [[nucleus]]
:* Peri-nuclear clearing ("water-clear rim" appearance)
:* Peri-nuclear clearing ("water-clear rim" appearance)
* A positive tartrate-resistant acid phosphatase (TRAP) stain is a key diagnostic feature for hairy cell leukemia.
* A positive tartrate-resistant acid phosphatase (TRAP) stain is a key diagnostic feature for hairy cell leukemia.
* On immunohistochemistry, characteristic findings of hairy cell leukemia include:
* On immunohistochemistry, characteristic findings of hairy cell leukemia include:
:* Annexin A1 +ve
:* [[Annexin A1]] +ve
:* CD20 +ve
:* [[CD20]] +ve
:* CD25 +ve
:* [[CD25]] +ve
:* CD103 +ve
:* [[CD103]] +ve
* On flow cytometry, characteristic findings of hairy cell leukemia include:
* On flow cytometry, characteristic findings of hairy cell leukemia include:
:* CD19 +ve
:* [[CD19]] +ve
:* CD11c +ve
:* [[CD11c]] +ve
:* FMC7 +ve
:* [[FMC7]] +ve
* Illustrated below is a series of microscopic images observed in hairy cell leukemia:
* Illustrated below is a series of microscopic images observed in hairy cell leukemia:
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Revision as of 19:22, 30 October 2015

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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 small cells with "fried egg"-like appearance, well-demarcated thread-like cytoplasmic extensions, and a clear cytoplasm.[4]

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]
  • Leukemic cells may also infiltrate both the spleen and liver.
  • Extravascular hemolysis may develop due to splenic sequestration of the circulating red blood cells.
  • In very rare occasions leukemic cells may infiltrate the peripheral lymph nodes.
  • Bone marrow failure may develop among hairy cell leukemia patients due to:[3]
  • The development of bone marrow failure interferes with the normal production of red blood cells and platelets among hairy cell leukemia patients.[5]
  • Production of cytokines, such as TNF alpha and IL-2R, provide important stimuli for malignant B cells proliferation in hairy cell leukemia.
  • Leukemic cells demonstrate prolonged survival due to the up-regulation of apoptosis-inhibitors such as IAP1 and IAP2 by TNF alpha.
  • In approximately 40% of hairy cell leukemia cases, malignant cells co-express multiple colonally related IgG, IgA, and IgM isotypes.

Genetics

  • The most common gene involved in the pathogenesis of hairy cell leukemia is BRAF V600E mutation.[3]
  • The BRAF V600E mutations is present among most of the patients with hairy cell leukemia (classic).
  • The BRAF V600E mutations is absent among patients with hairy cell leukemia (variant).
  • Molecular pathways involved in the pathogensis of hairy cell leukemia include:[5]
  • The p38-MAPK-JNK cascade is inhibited which will suppress the apoptotic signaling pathways.
  • The MEK-ERK cascade is activated which will amplify the cytoprotective survival pathways.
  • The Phosphatidylinositol 3 kinase (PI3K)-AKT cascade is activated thus suppressing the apoptotic signaling pathways.

Associated Conditions

  • Hairy cell leukemia has been found to be associated with trisomy 5 in a number of reported cases.[3]

Microscopic Pathology

  • On microscopic histopathological analysis, characteristic findings of hairy cell leukemia include:[4]
  • Small cells with "fried egg"-like appearance
  • Well-demarcated thread-like cytoplasmic extensions
  • Clear cytoplasm
  • Central round nucleus
  • Peri-nuclear clearing ("water-clear rim" appearance)
  • A positive tartrate-resistant acid phosphatase (TRAP) stain is a key diagnostic feature for hairy cell leukemia.
  • On immunohistochemistry, characteristic findings of hairy cell leukemia include:
  • On flow cytometry, characteristic findings of hairy cell leukemia include:
  • Illustrated below is a series of microscopic images observed in hairy cell leukemia:
  • Hairy cell leukemia illustrated on a blood film[4]

    Hairy cell leukemia illustrated on a blood film[4]

  • Hairy cell leukemia illustrated on high magnification[4]

    Hairy cell leukemia illustrated on high magnification[4]

  • Hairy cell leukemia illustrated on very high magnification[4]

    Hairy cell leukemia illustrated on very high magnification[4]

References

  1. ↑ 1.0 1.1 Magrath I. The Lymphoid Neoplasms 3ed. CRC Press; 2010.
  2. ↑ 2.0 2.1 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. ↑ 3.0 3.1 3.2 3.3 Hairy cell leukemia. Wikipedia (2015) https://en.wikipedia.org/wiki/Hairy_cell_leukemia#Pathophysiology Accessed on Ocotber, 19 2015
  4. ↑ 4.0 4.1 4.2 4.3 4.4 Hairy cell leukemia. Libre Pathology (2015) http://librepathology.org/wiki/index.php/Small_cell_lymphomas#Hairy_cell_leukemia Accessed on October, 8 2015
  5. ↑ 5.0 5.1 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.


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