Hairy Cell Leukemia Closely Related Entities

The mature-appearing lymphocytes of CLL may occasionally be confused with hairy cells, but are usually readily distinguished on the basis of morphology, immunophenotype (CD5 positivity and weak surface immunoglobulin staining), and cytochemistry (TRAP-negative) (Table 3-6). A hairy cell leukemia variant has been described217 in which the white count tends to be higher than in the typical disease, although almost all other features tend to be similar. In addition, the interleukin-2 receptor (CD25) is not expressed as commonly in the variant as it tends to be in straightforward cases. The clinical course of hairy cell leukemia variant tends to be aggressive.

Splenic lymphoma with villous lymphocytes233 is similar to hairy cell leukemia with prominent splenomegaly, but lym-phadenopathy is more common and bone marrow infiltration is less striking than in hairy cell leukemia. The malignant lymphocytes have thin, short, and unevenly distributed villi. The immunophenotypic features are variable with occasional CD5 positivity as in CLL and CD25 expression in hairy cell leukemia.234 The more clinically aggressive behavior of splenic lymphoma with villous lymphocytes may be accounted for, in some patients, by abnormalities of the p53 tumor suppressor gene,235 which occurs in about 20 percent. Lymphadenopathy is also common in monocytoid B-cell lymphoma, characterized by CD5+ small lymphocytes without projections and rare bone marrow involvement.236,237

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