Leukopenic chronic T cell leukemia mimicking hairy cell leukemia:
association with human retroviruses
CC Sohn, DW Blayney, JL Misset, G Mathe, G Flandrin, EM Moran, FC Jensen, CD Winberg and H Rappaport
We report two cases of a T cell lymphoproliferative disease not previously
described, with cytologic and clinical features similar to those associated
with Galton's "prolymphocytic" leukemia (PL). Our patients, like those with
Galton's PL, had massive splenomegaly and minimal or absent hepatomegaly
and lymphadenopathy. In contrast, however, our patients had leukopenia, as
well as low percentages of leukemic cells in the peripheral blood and in
the bone marrow. In splenic imprints, the nuclear chromatin pattern of most
of the leukemic cells was intermediate between those of mature lymphocytes
and those of lymphoblasts, and the nuclei contained single, centrally
located, conspicuous nucleoli. In sections of the spleen, the leukemic
cells diffusely infiltrated the red pulp in a pattern strikingly similar to
that of hairy cell leukemia; however, when the leukemic cells were studied
cytochemically, the cytoplasmic acid phosphatase positivity was punctate
and tartrate-sensitive. The leukemic cells were sheep erythrocyte
rosette-positive and expressed T cell-associated antigens. Initially, both
patients responded well to therapeutic splenectomy. One patient received
combination chemotherapy after splenectomy and is alive and well 24 months
after diagnosis. The other patient was in complete clinical remission for
one year after splenectomy and received chemotherapy at relapse. He died,
however, 23 months after splenectomy, with disseminated disease. IgG
antibody titers against human T lymphotropic virus type I (HTLV-I) were
detected in one patient and against HTLV-II in the other. The leukemia in
these patients represents a distinct clinicopathologic entity within the
spectrum of peripheral T cell lymphoproliferative diseases that includes
Galton's PL of T cell derivation, T cell chronic lymphocytic leukemia, T
cell hairy cell leukemia, and adult T cell leukemia/lymphoma.
Volume 67,
Issue 4,
pp. 949-956,
04/01/1986
Copyright © 1986 by The American Society of Hematology
