Chromosome abnormalities in hairy cell leukaemia variant

✍ Scribed by Dr. Vasantha Brito-Babapulle; Estela Matutes; David Oscier; Sarah Mould; Daniel Catovsky

• Book ID: 102846771
• Publisher: John Wiley and Sons
• Year: 1994
• Tongue: English
• Weight: 510 KB
• Volume: 10
• Category: Article
• ISSN: 1045-2257
• DOI: 10.1002/gcc.2870100308

✦ Synopsis

We describe chromosome abnormalities in 6 patients with hairy cell leukaemia (HCL) variant, a rare B-cell disorder with clinical and laboratory features intermediate between HCL and B-prolyrnphocytic leukaemia (6-PLL). All but one had marked splenomegaly and a raised white blood cell count (median 40 X 109/1) with over 80% nucleolated hairy cells. These cells had a 6-cell immunophenotype distinct from that of typical HCL. Alt patients but one are alive with stable disease with a median follow-up of 60 months. Numerical chromosome changes included IOU of chromosomes 2,3,4,6, 10. I9,21, and X. Three cases had translocations involving the immunoglobulin gene regions: t(14; 17)(q32;q I I), t( 14;22)(q32;ql I), and t(2;8)(p I I. 12;q24). lmmunocytochemistry demonstrated the presence of the MYC protein in cells from the case with t(2;8) but not in two others. Other structural abnormalities included t(3; IO)(q27;q22) and t(3; I 2)(q27;q I 3) in the same patient, der( I7)t(7; I 0 17) (pl l;q27;q22), t(l;3)(q25;pZI), t(8;21)(pl2;qI I), t(17;21)(pl I;pl I), de1(6)(q15), de1(7)(q34), and de1(14)(q24). Genes Chromosorn Cancer 10:197-202 (1994).