โ Scribed by Nnenna Osuji; Estella Matutes; Geir Tjonnfjord; Henri Grech; Ilaria Del Giudice; Andrew Wotherspoon; John G. Swansbury; Daniel Catovsky
No coin nor oath required. For personal study only.
To the authors' knowledge, there is no standard treatment for patients with Tโcell large granular lymphocyte (LGL) leukemia. Available data are limited by patient numbers and coexisting pathologies.
The authors report on the use of immunosuppressants (cyclosporin A [CSA] and lowโdose oral methotrexate [MTX] given continuously) and cytotoxic agents in the treatment of 29 patients with Tโcell LGL leukemia age over the past 20 years.
The overall response rate (ORR) to MTX (n = 8 patients) was 85.7% (complete hematologic response [CHR] rate, 14.3%; partial response [PR] rate, 71.4%) with doseโdependent responses observed and safe usage of doses >10 mg/m^2^ per week in 2 patients. The ORR to CSA (n = 23 patients) was 78.2% (CHR rate, 30.4%; PR rate, 47.8%). The median time to response for both agents was 1 month. Toxicity, although it was minor in most patients and was more common in the CSA group, included second malignancies in 5 patients. An ORR of 67% (all CHR) was attained with pentostatin (n = 4 patients); recurrences developed after a median of 4.6 years. Successful retreatment with pentostatin was possible but with increasing drug resistance. Cyclophosphamide induced CHR that lasted >7 years with bone marrow clearance in 1 of 4 patients. Alemtuzumab induced a PR in 1 patient who had refractory disease.
Both MTX and CSA were efficacious in the treatment of Tโcell LGL leukemia but generally required longโterm maintenance therapy. The authors highlight the risks of second malignancies and persistence of bone marrow disease. Although MTX and CSA were effective as firstโline therapy, alemtuzumab and pentostatin merit further investigation, particularly for refractory disease. Cancer 2006 ยฉ 2006 American Cancer Society.
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## Abstract The majority of patients with Tโcell large granular lymphocyte (LGL) leukemia will have an indolent clinical course. Herein, we report a case of an aggressive Tโcell LGL leukemia in a previously healthy 42โyearโold Caucasian male who presented with acute onset of Bโsymptoms, hepatosplen
Background. Granular cell tumor was first described by Abrikossoff in 1926. It is rare and usually presents as a benign solitary lesion. Multifocal and malignant forms are known to occur. Methods. This presentation illustrates an additional case of granular cell tumor. Clinical and histological fea