학술논문

Long-Smoldering T-prolymphocytic Leukemia: A Case Report and a Review of the Literature
Document Type
article
Source
Current Oncology, Vol 30, Iss 11, Pp 10007-10018 (2023)
Subject
T-prolymphocytic leukemia (T-PLL)
TCL1
ATM
JAK/STAT
alemtuzumab
pentostatin
Neoplasms. Tumors. Oncology. Including cancer and carcinogens
RC254-282
Language
English
ISSN
1718-7729
1198-0052
Abstract
T-prolymphocytic leukemia (T-PLL) is a rare malignancy of mature T-cells with distinct clinical, cytomorphological, and molecular genetic features. The disease typically presents at an advanced stage, with marked leukocytosis, B symptoms, hepatosplenomegaly, and bone marrow failure. It usually follows an aggressive course from presentation, and the prognosis is often considered dismal; the median overall survival is less than one year with conventional chemotherapy. This case report describes a patient with T-PLL who, after an unusually protracted inactive phase, ultimately progressed to a highly invasive, organ-involving disease. After initial treatments failed, a novel treatment approach resulted in a significant response.