This is the first report of a complete remission in aggressive T-cell large granular lymphocytic (
T-LGL) leukemia after treatment with
pentostatin. The aggressive variant of the disease is rare, and traditional
therapies include
immunosuppressive agents, however, there is no standard consensus for treatment. Cytotoxic
chemotherapy has led to remission in a few reported cases. We present this unique case as an alternative treatment for individuals refractory to
chemotherapy. A 55-year-old African American male with
hypertension, type II
diabetes mellitus,
hyperlipidemia, and
gout presented with symptoms of multiple
ecchymosis,
fatigue, and
weight loss. He was found to have
splenomegaly (SM) and significant
leukocytosis to 101 k/µL with 30% blasts on peripheral smear. Following bone marrow aspiration and biopsy with flow cytometry, he was diagnosed with aggressive
T-LGL leukemia. The
chemotherapy regimen hyper-CVAD (
cyclophosphamide,
vincristine,
doxorubicin, and
dexamethasone) was initially chosen based on his clinical presentation but was refractory to treatment. His
therapy was changed to
alemtuzumab; however, patient tolerated poorly and did not respond.
Pentostatin was added to
alemtuzumab with improvement in clinical symptoms and laboratory parameters. The patient was transitioned to
pentostatin monotherapy and achieved complete remission after 1 month. This report provides support for
pentostatin as an effective treatment for patients with aggressive T-cell
malignancies refractory to cytotoxic
chemotherapy.
Pentostatin has previously been studied to treat
T-cell prolymphocytic leukemia (T-PLL),
hairy cell leukemia, and marginal zone
lymphoma. This case suggests an alternative, well-tolerated option that could be considered for initial
therapy of aggressive
T-LGL leukemia.