Abstract | INTRODUCTION: Large granular lymphocyte ( LGL) leukemia is a rare chronic lymphoproliferative disorder, with few large series reported to date. Series using stringent diagnostic criteria incorporating bone marrow biopsy (BMB), immunophenotyping, and T-cell receptor rearrangements are even scarcer. PATIENTS AND METHODS: The present study was a single-center series of 39 patients with LGL leukemia diagnosed using immunohistochemical analysis of BMB samples and flow cytometric and molecular data. RESULTS: With a median follow-up of 3.2 years (range, 1.0-15.1 years), 15 patients (38%) never required treatment. Of the remaining 24 patients requiring treatment, 13 were initially treated with prednisolone, for an overall response rate (ORR) of 84.6% and a median duration of response (DOR) of 13.5 months (range, 5.7-70.3 months). Of the 24 patients, 9 received oral low-dose weekly methotrexate as first-line therapy, with 8 (89%) achieving a hematologic response and a median DOR of 132.7 months (range, 6.7-180.5 months). Another 5 patients received methotrexate after prednisolone failure; all 5 responded, with a median DOR of 14 months (range, 4-96 months). Only 2 patients developed progression during methotrexate therapy, and 4 patients experienced responses lasting ≥ 5 years. CONCLUSION: Single-agent oral methotrexate appears to be highly efficacious, resulting in long response durations and minimal toxicity.
|
Authors | Talha Munir, Mark J Bishton, Ian Carter, Andrew McMillan, Simon O'Connor, Vishakha Sovani, Andrew P Haynes, Christopher P Fox |
Journal | Clinical lymphoma, myeloma & leukemia
(Clin Lymphoma Myeloma Leuk)
Vol. 16
Issue 12
Pg. 705-712
(12 2016)
ISSN: 2152-2669 [Electronic] United States |
PMID | 27640075
(Publication Type: Journal Article)
|
Copyright | Crown Copyright © 2016. Published by Elsevier Inc. All rights reserved. |
Chemical References |
- Antimetabolites, Antineoplastic
- Methotrexate
|
Topics |
- Administration, Oral
- Adult
- Aged
- Aged, 80 and over
- Antimetabolites, Antineoplastic
(administration & dosage)
- Biopsy
- Bone Marrow
(pathology)
- Female
- Follow-Up Studies
- Gene Rearrangement, T-Lymphocyte
- Humans
- Immunophenotyping
- Leukemia, Large Granular Lymphocytic
(diagnosis, drug therapy, metabolism, mortality)
- Male
- Methotrexate
(administration & dosage)
- Middle Aged
- Survival Analysis
- Treatment Outcome
- Young Adult
|