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Clinical spectrum of clonal proliferations of T-large granular lymphocytes:
a T-cell clonopathy of undetermined significance?
MV Dhodapkar, CY Li, JA Lust, A Tefferi and RL Phyliky
Division of Hematology, Mayo Clinic and Foundation, Rochester, MN 55905.
We identified 68 patients with clonal T-large granular lymphocyte (T- LGL)
proliferations who were seen at the Mayo Clinic between 1984 and 1992.
Nineteen (28%) were asymptomatic at diagnosis, while the rest experienced
fatigue (60%), B-symptoms (12%), and recurrent infections (15%). Associated
comorbid conditions included rheumatoid arthritis (RA) in 26%. Severe
anemia (hemoglobin [Hb] < 8g/dL) and neutopenia (absolute neutrophil
count [ANC] < 500/microL) were seen in 19% and 40% of patients,
respectively. Immunophenotypic studies showed CD3+, CD8+ phenotype in the
majority (72%). Twenty-one patients (31%) have required no therapy, and
remain relatively stable with a median follow- up period of 50 months.
Treatment was required at either diagnosis (36 patients) or at subsequent
follow-up (11 patients). Initial response rates were similar in patients
treated with cyclophosphamide (CTX) with or without prednisone (69%), or
prednisone alone (73%). Overall, 61 patients (90%) are alive with a median
follow-up of 44 months. Actuarial median survival of this entire cohort is
161 months. The presence of anemia or symptoms does not appear to correlate
with the tumor burden. In patients requiring therapy, a lower ANC and the
presence of B-symptoms/infection were independently associated with a
significantly lower probability of achieving a molecular or hematologic
complete remission (H-CR). Intermittent immunosuppressive therapy is
effective in achieving durable responses in a number of patients. T-LGL
proliferations are associated with a favorable prognosis and response to
therapy. However, significant heterogeneity exists in clinical presentation
and associated comorbid conditions. These disorders should be included in
the differential diagnosis of patients with unexplained cytopenias,
particularly in the setting of RA and other autoimmune disorders. Analogous
to the situation with monoclonal gammopathies, a term such as T-cell
clonopathy of undetermined significance (TCUS) may be more appropriate to
describe these patients.
Volume 84,
Issue 5,
pp. 1620-1627,
09/01/1994
Copyright © 1994 by The American Society of Hematology
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