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Difference between revisions of "Hairy cell leukemia"
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[[File:Bone marrow infiltration by hairy cell leukaemia.jpg|thumb|High power magnification of a bone marrow trephine showing infiltration by hairy cell leukaemia.]] | [[File:Bone marrow infiltration by hairy cell leukaemia.jpg|thumb|High power magnification of a bone marrow trephine showing infiltration by hairy cell leukaemia.]] | ||
Indolent B-cell neoplasm, abbreviated ''HCL''. Named for the appearances on peripheral blood smears. | Indolent B-cell neoplasm, abbreviated ''HCL''. Named for the appearances on peripheral blood smears. The vast majority (>95%) harbour the BRAF V600E mutation.<ref name=pmid25992240>{{Cite journal | last1 = Ahmadzadeh | first1 = A. | last2 = Shahrabi | first2 = S. | last3 = Jaseb | first3 = K. | last4 = Norozi | first4 = F. | last5 = Shahjahani | first5 = M. | last6 = Vosoughi | first6 = T. | last7 = Hajizamani | first7 = S. | last8 = Saki | first8 = N. | title = BRAF Mutation in Hairy Cell Leukemia. | journal = Oncol Rev | volume = 8 | issue = 2 | pages = 253 | month = Sep | year = 2014 | doi = 10.4081/oncol.2014.253 | PMID = 25992240 }}</ref> Mutations in other parts of the BRAF gene are described, but rare.<ref name=pmid24433452>{{Cite journal | last1 = Tschernitz | first1 = S. | last2 = Flossbach | first2 = L. | last3 = Bonengel | first3 = M. | last4 = Roth | first4 = S. | last5 = Rosenwald | first5 = A. | last6 = Geissinger | first6 = E. | title = Alternative BRAF mutations in BRAF V600E-negative hairy cell leukaemias. | journal = Br J Haematol | volume = 165 | issue = 4 | pages = 529-33 | month = May | year = 2014 | doi = 10.1111/bjh.12735 | PMID = 24433452 }}</ref> | ||
In the bone marrow, they are associated with heavy reticulin fibrosis, commonly resulting in ''dry taps''.<ref name=pmid22499303>{{Cite journal | last1 = Galani | first1 = KS. | last2 = Subramanian | first2 = PG. | last3 = Gadage | first3 = VS. | last4 = Rahman | first4 = K. | last5 = Ashok Kumar | first5 = MS. | last6 = Shinde | first6 = S. | last7 = Mahadik | first7 = S. | last8 = Ansari | first8 = R. | last9 = Sengar | first9 = M. | title = Clinico-pathological profile of Hairy cell leukemia: critical insights gained at a tertiary care cancer hospital. | journal = Indian J Pathol Microbiol | volume = 55 | issue = 1 | pages = 61-5 | month = | year = | doi = 10.4103/0377-4929.94858 | PMID = 22499303 }}</ref> | |||
Clinical:<ref>URL: [http://www.ncbi.nlm.nih.gov/bookshelf/br.fcgi?book=cmed&part=A34022 http://www.ncbi.nlm.nih.gov/bookshelf/br.fcgi?book=cmed&part=A34022]. Accessed on: 20 August 2010.</ref> | Clinical:<ref>URL: [http://www.ncbi.nlm.nih.gov/bookshelf/br.fcgi?book=cmed&part=A34022 http://www.ncbi.nlm.nih.gov/bookshelf/br.fcgi?book=cmed&part=A34022]. Accessed on: 20 August 2010.</ref> | ||
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*Good prognosis (with treatment), though (likely) not curable. | *Good prognosis (with treatment), though (likely) not curable. | ||
==Gross== | |||
Features:<ref name=Ref_PCPBoD8_326>{{Ref PCPBoD8|326}}</ref> | Features:<ref name=Ref_PCPBoD8_326>{{Ref PCPBoD8|326}}</ref> | ||
*Huge beefy red [[spleen]]. | *Huge beefy red [[spleen]]. | ||
**Red as white pulp obliterated. | **Red as white pulp obliterated. | ||
==Microscopic== | |||
Features:<ref>URL: [http://emedicine.medscape.com/article/200580-diagnosis http://emedicine.medscape.com/article/200580-diagnosis]. Accessed on: 18 August 2010.</ref> | Features:<ref>URL: [http://emedicine.medscape.com/article/200580-diagnosis http://emedicine.medscape.com/article/200580-diagnosis]. Accessed on: 18 August 2010.</ref> | ||
*Small cells (10-20 micrometers) with "Fried egg"-like appearance: | *Small cells (10-20 micrometers) with "Fried egg"-like appearance: | ||
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*[[Hepatosplenic T-cell lymphoma]]. | *[[Hepatosplenic T-cell lymphoma]]. | ||
===Images=== | |||
<gallery> | <gallery> | ||
Image:Hairy_cell_leukemia.jpg | HCL - blood film. (WC) | Image:Hairy_cell_leukemia.jpg | HCL - blood film. (WC) | ||
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*[http://webpathology.com/image.asp?case=380&n=3 HCL in the spleen (webpathology.com)]. | *[http://webpathology.com/image.asp?case=380&n=3 HCL in the spleen (webpathology.com)]. | ||
==IHC== | |||
Features:<ref>URL: [http://www.ncbi.nlm.nih.gov/bookshelf/br.fcgi?book=cmed&part=A34022&rendertype=table&id=A34029 http://www.ncbi.nlm.nih.gov/bookshelf/br.fcgi?book=cmed&part=A34022&rendertype=table&id=A34029]. Accessed on: 20 August 2010.</ref> | Features:<ref>URL: [http://www.ncbi.nlm.nih.gov/bookshelf/br.fcgi?book=cmed&part=A34022&rendertype=table&id=A34029 http://www.ncbi.nlm.nih.gov/bookshelf/br.fcgi?book=cmed&part=A34022&rendertype=table&id=A34029]. Accessed on: 20 August 2010.</ref> | ||
*CD20 +ve, CD11c+ve, CD25 +ve, CD103 +ve, CD123 +ve, cyclin D1 +ve, DBA44 +ve | *CD20 +ve, CD11c+ve, CD25 +ve, CD103 +ve, CD123 +ve, cyclin D1 +ve, DBA44 +ve | ||
*Annexin A1 +ve (only useful in heavy bone marrow infiltration as it is also positive in T-cells and granulopoietic cells) | |||
*TRAP - tartrate-resistant acid phosphatase (historically histochemically, now immunohistochemically) | |||
*[[HBME1]]<ref name=pmid24092261>{{cite journal |authors=Krenács L, Tóth-Lipták J, Demeter J, Piukovics K, Borbényi Z, Gogolák P, Sári E, Bagdi E |title=Monoclonal antibody HBME-1 reacts with a minor subset of B cells with villous surface and can be useful in the diagnosis of hairy cell leukemia and other indolent lymphoproliferations of villous B lymphocytes |journal=Virchows Arch. |volume=463 |issue=6 |pages=787–94 |date=December 2013 |pmid=24092261 |doi=10.1007/s00428-013-1490-5 |url=}}</ref> | |||
*BRAF V600E mutation-specific antibody | |||
*CD5 -ve. | *CD5 -ve. | ||
Flow cytometry: | Flow cytometry: | ||
*CD19 +ve, CD11c +ve, FMC7 +ve. | *CD19 +ve, CD11c +ve, FMC7 +ve. | ||
==See also== | |||
*[[Small cell lymphomas]]. | |||
==References== | |||
{{Reflist}} | |||
[[Category: Haematopathology]] | |||