We present a case of lymphocytosis assumed and managed initially as a chronic lymphocytic leukemia. Shortly after initial visit, the patient's condition deteriorated rapidly with hepatosplenomegaly, pleural effusion, ascites, and skin lesions. Flow cytometry (FC) showed the presence of clonal T-cell population, reported as T-cell lymphoma. Due to rapid clinical deterioration, urgent therapy with cyclophosphamide, doxorubicin, vincristine, etoposide, prednisone was initiated, but with minimal response. This prompted further diagnostic testing and demonstrated tumor cells positivity for CD3, CD30, and TCL1 markers. The diagnosis was changed to T-cell prolymphocytic leukemia. The patient responded well to alemtuzumab (anti-CD52 monoclonal antibody) and reached complete remission. FC is an essential modality for assessing and screening circulating lymphocytes when a lymphoproliferative disorder (LPD) is suspected. There are several LPDs that present with different degrees of clonal lymphocytosis. Reactive lymphocytosis should be appropriately investigated. Indolent LPDs can be surveyed by the internist or family physician, while more aggressive LPDs typically require management by hematologists.
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http://dx.doi.org/10.1159/000531592 | DOI Listing |
Hematology
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.
View Article and Find Full Text PDFCase Rep Hematol
August 2024
Department of Oncology, Hematology, Immune-Oncology and Rheumatology University Hospital Bonn, Bonn, Germany.
T-prolymphocytic leukaemia (T-PLL) is the most common mature T-cell leukaemia in Central Europe and is often manifested by rapidly increasing lymphocytosis, marked bone marrow infiltration and splenomegaly. In 10-15% of cases, the diagnosis is made by incidental findings in otherwise asymptomatic patients. Here we report a case of T-PLL that initially became symptomatic due to the presence of acute coronary syndrome (ACS).
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