T-cell prolymphocytic leukemia is a rare postthymic malignancy with distinctive clinical, morphologic, immunophenotypic, and cytogenetic features. The clinical course is typically aggressive with poor response to conventional chemotherapy and short survival. Treatment with purine analogues and alemtuzumab has resulted in significantly higher response rates and improved survival. Nevertheless, responses are relatively short, and the only potential curative treatment is allogeneic stem cell transplantation. The age and comorbidities of many of the patients has limited this option, but the growing use of nonmyeloablative transplantation has now widened the patient eligibility for this approach.
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http://dx.doi.org/10.3816/CLM.2009.s.018 | DOI Listing |
Int J Mol Sci
December 2024
Hematology Section, Department of Medicine, Hematology and Clinical Immunology Branch, University of Padova, 35122 Padova, Italy.
Finding an effective treatment for T-PLL patients remains a significant challenge. Alemtuzumab, currently the gold standard, is insufficient in managing the aggressiveness of the disease in the long term. Consequently, numerous efforts are underway to address this unmet clinical need.
View Article and Find Full Text PDFHematology
December 2025
National Clinical Research Center for Hematologic Diseases, Jiangsu Institute of Hematology, The First Affiliated Hospital of Soochow University, Soochow University, Suzhou, People's Republic of China.
Objective: Hematological malignancies often involve chromosomal translocations and fusion genes that drive disease progression. While is well-known in T-cell prolymphocytic leukemia (T-PLL), its role in myeloid neoplasms is less understood. This report presents the first identification of the t(X;1)(q28;q21) translocation leading to the fusion in acute myeloid leukemia (AML) transformed from chronic myelomonocytic leukemia (CMML).
View Article and Find Full Text PDFJMA J
October 2024
Department of Hematology, Fukushima Medical University Aizu Medical Center, Aizuwakamatsu, Japan.
T-cell prolymphocytic leukemia (T-PLL) is a rare and highly aggressive mature T-cell neoplasm. Although the response rate to alemtuzumab, an anti-CD52 antibody, is high, it is difficult to cure the disease with this agent alone. Therefore, hematopoietic stem cell transplantation is recommended for eligible patients.
View Article and Find Full Text PDFAnn Hematol
November 2024
Dept. Internal Medicine C, Haematology, Oncology, Stem Cell Transplantation, University Hospital Greifswald, Palliative Care. Ferdinand Sauerbruch Street, 17475, Greifswald, Germany.
T-Cell Prolymphocytic Leukaemia (T-PLL) is an aggressive disease with a poor prognosis and only curable by allogeneic stem cell transplantation. We describe the case of a male suffering from T-PLL. Therapy was alemtuzumab followed by an allograft from an unrelated donor.
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