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hairy cell leukemia
Saturday 5 September 2009
Tricholeukocyte leukemia; HCL
Images
Hairy cell leukemia. Pancytopenia w/ monocytopenia. CD11c+ /CD25+ /CD103+ /CD123+ . BRAF-V600E mut. 10yr survival >90%.
Digital slides
Descartes:9667 (ICSFramework)
HPC:281 : Hairy cell leukemia in bone marrow
Cytogenetics
Overrepresentation of chromosome 5 represents a recurrent aberration in HCL and that the commonly overrepresented region resides in 5q13-q31.
Molecular biology
The somatically acquired V600E mutation of the BRAF gene has been recently described as a molecular marker of hairy cell leukemia (HCL).
BRAF-V600E mutation is present in all HCL patients and that, in combination with clinical and morphological features, represents a reliable molecular marker for this condition. (22072557)
References
Phospho-ERKTHR202/Tyr214 Is Overexpressed in Hairy Cell Leukemia and Is a Useful Diagnostic Marker in Bone Marrow Trephine Sections. Warden DW, Ondrejka S, Lin J, Durkin L, Bodo J, Hsi ED. Am J Surg Pathol. 2012 Dec 1. PMID: 23211289
T-bet transcription factor detection facilitates the diagnosis of minimal hairy cell leukemia infiltrates in bone marrow trephines. Jöhrens K, Stein H, Anagnostopoulos I. Am J Surg Pathol. 2007 Aug;31(8):1181-5. PMID: 17667540
The BRAF V600E mutation in hairy cell leukemia and other mature B-cell neoplasms. Arcaini L, Zibellini S, Boveri E, Riboni R, Rattotti S, Varettoni M, Guerrera ML, Lucioni M, Tenore A, Merli M, Rizzi S, Morello L, Cavalloni C, Da Vià MC, Paulli M, Cazzola M. Blood. 2011 Nov 9. PMID: 22072557
Chromosomal imbalances including deletions of the tumor suppressor gene loci 9p21/P16(INK4A), 13q14/D13S25, and 17p13/P53 rarely occur in HCL in contrast to some other subtypes of B-cell NHL. (11463458)
Chromosomal gains and losses are uncommon in hairy cell leukemia: a study based on comparative genomic hybridization and interphase fluorescence in situ hybridization. Dierlamm J, Stefanova M, Wlodarska I, Michaux L, Hinz K, Penas EM, Maes B, Hagemeijer A, De Wolf-Peeters C, Hossfeld DK. Cancer Genet Cytogenet. 2001 Jul 15;128(2):164-7. PMID: 11463458
