• Diagn Pathol · Jul 2019

    Case Reports

    Panniculitis-like T-cell-lymphoma in the mesentery associated with hemophagocytic syndrome: autopsy case report.

    • Jan Hrudka, Václav Eis, Josef Heřman, Zuzana Prouzová, Andreas Rosenwald, and František Duška.
    • Department of Pathology, Charles University, 3rd Faculty of Medicine, Charles University and Kralovske Vinohrady University Hospital, Prague, Czech Republic. jan.hrudka@lf3.cuni.cz.
    • Diagn Pathol. 2019 Jul 17; 14 (1): 80.

    BackgroundPanniculitis-like T-cell lymphoma is an uncommon type of non-Hodgkin lymphoma, occurring usually in the form of nodules within the subcutaneous fat tissue of the extremities or trunk. In the literature, subcutaneous panniculitis-like T-cell lymphoma (SPTCL) is described as a distinct type of T-cell lymphoma with a variable clinical behavior, depending on molecular phenotype of T-cell receptor (TCR) and on the presence or absence of hemophagocytic syndrome.Case PresentationWe present a bioptic and autoptic case of a 65-years old Caucasian man with panniculitic T-cell lymphoma with morphological and immunohistochemical features of SPTCL, limited to the retroperitoneal and mesenteric mass, i.e. without any cutaneous involvement, and associated with severe hemophagocytic lymphohistiocytosis.ConclusionA panniculitic T-cell lymphoma with morphological and molecular features of SPTCL, which is limited to mesentery, i.e. does not involve subcutaneous fat, seems to be exceedingly rare.

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