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Blood, 15 November 2000, Vol. 96, No. 10, pp. 3644-3646
BRIEF REPORT
Lymphoproliferative disease of granular T lymphocytes
presenting as aplastic anemia
Ronald S. Go,
Ayalew Tefferi,
Chin-Yang Li,
John A. Lust, and
Robert L. Phyliky
From the Divisions of Hematology and Hematopathology,
Mayo Clinic and Foundation, Rochester, MN.
Lymphoproliferative disease of granular T lymphocyte (T-LDGL), also
known as T-cell large granular lymphocyte leukemia, is a clonal
disorder of cytotoxic T lymphocytes that is clinically manifested as
chronic neutropenia and anemia. Association with autoimmune disorders
is common. In 9 patients, T-LDGL is reported as presenting as aplastic
anemia. The clinical characteristics were similar to acquired aplastic
anemia. Morphologic evidence of increased granular lymphocytes in the
peripheral blood and an excess of
CD3+/CD8+/CD57+ cells in the bone
marrow were found in most cases. Cyclophosphamide was ineffective, but
noncytotoxic immunosuppressive agents generally produced a good
response. After a median follow-up of 49 months, 5 patients had died
from the disease or related complications. Median survival was 40 months. Aplastic anemia can be a presenting manifestation of T-LDGL,
and T-LDGL should be considered in the differential diagnosis of
acquired aplastic anemia.
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