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RW McKenna, DC Arthur, KJ Gajl-Peczalska, P Flynn and RD Brunning
The clinical, morphological, immunologic, and cytogenetic features of seven
cases of chronic granulated T cell lymphocytosis with neutropenia were
studied. The disorder was characterized by moderate blood and bone marrow
lymphocytosis, neutropenia, polyclonal hypergammaglobulinemia,
splenomegaly, absence of lymphadenopathy, and a chronic, relatively stable
clinical course. The proliferative lymphocytes manifested a
cytotoxic/suppressor T lymphocyte phenotype. In two of four cases studied,
blood lymphocytes showed clonal chromosome abnormalities. One patient
treated with pulse steroid therapy had reversal of lymphocytosis and severe
neutropenia with subsequent resolution of an intractable infection. The
lymphocytosis and neutropenia recurred when steroids were withdrawn. Six of
the seven patients were living three months to 17 years from diagnosis; one
died at 4.3 years of an unrelated cause. Five of the patients, including
the two with lymphocyte chromosome abnormalities, had persistent
lymphocytosis and neutropenia from three months to 13 years from diagnosis.
In two patients, the disease appears to have undergone spontaneous
regression. No differences in clinical presentation or the morphological or
immunologic characteristics of the proliferative lymphocytes were apparent
between those patients with lymphocyte chromosome abnormalities and
persistent disease and those who had a spontaneous regression. The finding
of clonal chromosome abnormalities in the blood lymphocytes of two of the
patients in this study suggests a neoplastic origin for chronic granulated
T cell lymphocytosis with neutropenia. However, apparent spontaneous
regression in two patients, one after 11 years, lends support to a chronic
reactive or immunoregulatory disorder as the etiology. It is probable that
cases of granulated T cell lymphocytosis with neutropenia, although
morphologically and immunologically similar, are biologically
heterogeneous.
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| Copyright © 1985 by American Society of Hematology Online ISSN: 1528-0020 | |||||||||