T-cell large granular lymphocytic leukemia: treatment experience with fludarabine

Abstract

OBJECTIVES:The aim of this retrospective study was to investigate the results of T-cell large granular lymphocytic leukemia treatment with fludarabine by assessing the complete hematologic response, the complete molecular response, progression-free survival, and overall survival.METHODS:We evaluated the records of six patients with T-cell large granular lymphocytic leukemia who were treated with fludarabine as a first-, second-, or third-line therapy, at a dose of 40 mg/m2, for three to five days per month and 6 to 8 cycles.RESULTS:Of the six patients investigated with T-cell large granular lymphocytic leukemia who were treated with fludarabine, five (83.3%) were female, and their median age was 36.5 years (range 18 to 73). The median lymphocyte level was 3.4×109/L (0.5 to 8.9). All patients exhibited a monoclonal T-cell receptor gamma gene rearrangement at diagnosis. Two (33.3%) patients received fludarabine as first-line treatment, two (33.3%) for refractory disease, one (16.6%) for relapsed disease after the suspension of methotrexate treatment due to liver toxicity, and one (16.6%) due to dyspesia. A complete hematologic response was achieved in all cases, and a complete molecular response was achieved in five out six cases (83.3%). During a mean follow-up period of 12 months, both the progression-free survival and overall survival rates were 100%.CONCLUSION:T-cell large granular lymphocytic leukemia demonstrated a high rate of complete hematologic and molecular response to fludarabine, with excellent compliance and tolerability rates. To confirm our results in this rare disease, we believe that fludarabine should be tested in clinical trials as a first-line treatment for T-cell large granular lymphocytic leukemia.

T-Cell Large Granular Lymphocytic Leukemia as a Cause for Severe Neutropenia

“…There are varying methods of disease treatment, which includes chemotherapy, immunotherapy or hormonal therapy, and combinations of these therapies. Among patients requiring treatment, which is approximately 60%, the overall survival at five years is approximately 62% [7,8]. Among all patients diagnosed with LGL leukemia, the overall survival at 10 years is approximately 70% [1].…”

Moderate-dose cyclophosphamide in the treatment of relapsed/refractory T-cell large granular lymphocytic leukemia-associated pure red cell aplasia

“…alemtuzumab, anti-CD52, and the humanized MiK-beta1 monoclonal antibody), combination chemotherapy (CTX, doxorubicin, vincristine, prednisolone [CHOP]), and splenectomy have all been used in refractory/relapsed and treatment-naïve patients, but the response rates of these agents vary between studies. [19][20][21][22][23][24][25] Most physicians consider that high-dose combination chemotherapy regimens are relatively ineffective and are associated with more toxicity than benefits for this indolent disorder. Instead, chronic exposure to low-dose therapy seems more efficient than sequential high-dose combination chemotherapy, despite some patients being refractory to single oral MTX or CTX.…”

CD20-Positive T-Cell Large Granular Lymphocytic Leukemia: A Case Report and Literature Review

“…In the second patient, rituximab therapy was followed by immediate neutropenia recovery; then, one year later, the LGLL clone showed marked shrinkage [21]. Raposo et al and Verhoevan et al also described one and three cases, respectively, where rituximab therapy improved rheumatoid arthritis control and increased the neutrophil count, with sustained responses [20][21][22]. In the four cases of CD20+…”

“…Rituximab treatment was described in a few cases of T-LGLL, which were associated with rheumatoid arthritis, to control the autoimmune aspects of the disease [20][21][22]. In general, good responses were achieved with a monoclonal antibody against CD20.…”