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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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) | |||
*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. | ||
===References=== | |||
{{Reflist}} | |||
[[Category: Haematopathology]] | |||