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

. 2019 Jul 17 ; 14 (1) : 80. [epub] 20190717

Language English Country England, Great Britain Media electronic

Document type Case Reports, Journal Article

Grant support
PROGRES Q28 Oncology Univerzita Karlova v Praze
Q37 Univerzita Karlova v Praze

Links

PubMed 31311562
PubMed Central PMC6636044
DOI 10.1186/s13000-019-0854-9
PII: 10.1186/s13000-019-0854-9
Knihovny.cz E-resources

BACKGROUND: Panniculitis-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 PRESENTATION: We 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. CONCLUSION: A 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.

See more in PubMed

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