T-cell prolymphocytic leukemia is a lymphoid hematologic malignancy of T origin, mature, individualized well by the clinical, morphological, and immunophenotypic as well as cytogenetic aspects. We report the case of a 39-year-old man admitted in our department because of a spinal cord compression. The cerebrospinal fluid analysis had revealed an increased albuminemia level; MRI evoked a vertebral infiltration by a malignancy. Evolution was characterized by the appearance of a tumor syndrome and a peripheral lymphocytosis. Immunophenotyping and the lymph node biopsy concluded of a diagnosis of a T-cell prolymphocytic leukemia. To our knowledge, we report here the first case of T-cell prolymphocytic leukemia revealed by a spinal cord compression.
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http://dx.doi.org/10.1684/abc.2013.0792 | DOI Listing |
Hematol Rep
January 2025
Department of 1st Internal Medicine, Medical School, University of Pécs, Ifjúság Str. 13, 7624 Pécs, Hungary.
T-cell prolymphocytic leukemia (T-PLL) is a rare mature T-cell lymphoma that is usually associated with poor prognosis and short overall survival. We present a case of a 61-year-old woman presenting with T-PLL and the leukemic cells harboring (-breakpoint cluster region; -ABL protooncogene 1) fusion transcripts as the result of a variant of t(9;22)(q34;q11) called Philadelphia translocation: t(9;22;18)(q34;q11;q21). Sequencing revealed a rare transcript with an exon 6 breakpoint corresponding to e6a2 transcripts, which has thus far been reported in only 26 cases of leukemias.
View Article and Find Full Text PDFInt J Lab Hematol
January 2025
Department of Pathology, University of Southern California Keck School of Medicine, Los Angeles, California, USA.
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).
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