Autor: |
Gjelberg HK; Department of Pathology, Haukeland University Hospital, N-5021 Bergen, Norway., Helgeland L; Department of Pathology, Haukeland University Hospital, N-5021 Bergen, Norway.; Department of Clinical Science, University of Bergen, N-5021 Bergen, Norway., Liseth K; Department of Immunology and Transfusion Medicine, Haukeland University Hospital, N-5021 Bergen, Norway., Micci F; Section for Cancer Cytogenetics, Institute of Cancer Genetics and Informatics, Oslo University Hospital, N-0424 Oslo, Norway., Sandnes M; Department of Medicine, Haukeland University Hospital, N-5021 Bergen, Norway., Russnes HG; Department of Pathology, Oslo University Hospital, N-0424 Oslo, Norway.; Department of Cancer Genetics, Institute for Cancer Research, Oslo University Hospital, N-0424 Oslo, Norway.; Institute for Clinical Medicine, Faculty of Medicine, University of Oslo, N-0424 Oslo, Norway., Reikvam H; Department of Medicine, Haukeland University Hospital, N-5021 Bergen, Norway.; Department of Medical Science, University of Bergen, N-5021 Bergen, Norway. |
Abstrakt: |
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. |