Panniculitis-like T-cell-lymphoma in the mesentery associated with hemophagocytic syndrome: autopsy case report
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
PubMed
31311562
PubMed Central
PMC6636044
DOI
10.1186/s13000-019-0854-9
PII: 10.1186/s13000-019-0854-9
Knihovny.cz E-resources
- Keywords
- Hemophagocytosis, Lymphohistiocytosis, Mesentery, Panniculitis, T-cell lymphoma,
- MeSH
- Diagnosis, Differential MeSH
- Lymphoma, T-Cell, Cutaneous diagnosis pathology MeSH
- Humans MeSH
- Lymphohistiocytosis, Hemophagocytic diagnosis pathology MeSH
- Lymphoma, T-Cell diagnosis pathology MeSH
- Skin Neoplasms diagnosis pathology MeSH
- Panniculitis diagnosis pathology MeSH
- Autopsy MeSH
- Aged MeSH
- T-Lymphocytes pathology MeSH
- Check Tag
- Humans MeSH
- Male MeSH
- Aged MeSH
- Publication type
- Journal Article MeSH
- Case Reports MeSH
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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