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A variant form of hairy cell leukemia resistant to alpha-interferon:
clinical and phenotypic characteristics of 17 patients
L Sainati, E Matutes, S Mulligan, MP de Oliveira, S Rani, IA Lampert and D Catovsky
Academic Department of Haematology and Cytogenetics, Royal Marsden
Hospital, London, UK.
We describe the clinical and laboratory features of 17 adult patients with
a variant form of hairy cell leukemia (HCL-V) studied over the last 7
years. The main findings were: splenomegaly, moderate anemia,
thrombocytopenia, and a raised white blood cell count (median 116 x
10(9)/L; range 15 to 482). The circulating lymphoid cells had abundant
villous cytoplasm and a round, occasionally bilobed nucleus, with a
prominent nucleolus. Monocytopenia, a feature of typical HCL, was not seen;
neither was tartrate-resistant acid phosphatase demonstrated in eight cases
tested. HCL-V cells had a mature B-cell phenotype: CD19+, CD20+, CD22+,
FMC7+, CD11c+, CD10-, CD5-, with light chain isotope restriction in 15
cases. In contrast to typical hairy cells, HCL-V cells were negative with
the monoclonal antibodies anti-HC2 and anti- TAC (CD25). Immunoglobulin
(Ig) was not detected in two cases and IgG was expressed in the cell
membrane of 73% of cases. Bone marrow histology was different from HCL,
showing interstitial infiltration by cells clumped together and a moderate
amount of reticulin, but the spleen showed the typical red pulp expansion
of HCL. HCL-V patients did not respond to splenectomy (5 of 7) or
alpha-interferon (7 of 7); 2 of 3 patients had a partial response to
2'deoxycoformycin. The clinical course was benign with 15 patients alive
with a median survival greater than 4 years. We confirm that HCL-V is a
distinct clinico-pathologic entity with intermediate features between HCL
and B-prolymphocytic leukemia.
Volume 76,
Issue 1,
pp. 157-162,
07/01/1990
Copyright © 1990 by The American Society of Hematology
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