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Case Reports
. 2019 Jul 17;14(1):80.
doi: 10.1186/s13000-019-0854-9.

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

Jan Hrudka  1 , Václav Eis  2 , Josef Heřman  2 , Zuzana Prouzová  3 , Andreas Rosenwald  4 , František Duška  5
Affiliations
Case Reports

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

Jan Hrudka et al. Diagn Pathol. .

Abstract

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.

Keywords: Hemophagocytosis; Lymphohistiocytosis; Mesentery; Panniculitis; T-cell lymphoma.

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Conflict of interest statement

The authors declare that they have no competing interests.

Figures

Fig. 1
Fig. 1
CT scan showing an infiltrate in mesocolon ascendens invading radix mesenterii and the mesenteric vessels
Fig. 2
Fig. 2
scans of histological slides from the mesenterial fat tissue showing dense lymphocytic infiltration consisting mostly of medium-sized cells with hyperchromatic irregular nuclei. Note a pattern of isolated adipocytes surrounded by a rim of lymphocytes (“rimming”) and large macrophages engulfing lymphocytes (hemophagocytosis). A – HE 60x, B – Giemsa 85x, C – Giemsa 105x, D – HE 145x
Fig. 3
Fig. 3
immunohistochemistry showing neoplastic lymphocytes with positivity of CD3, CD8, perforin, TIA-1 and TCRβF1. CD4 stains rather bystander T-cells and histiocytes, without unequivocal positivity in lymphoma. The neoplastic cells are CD20 negative, note the sparse CD20-positive reactive B-cells. The neoplastic lymphocytes embody proliferation activity (Ki67) about 50%
Fig. 4
Fig. 4
scans of histological slides from the autopsy. A – vertebral bone marrow with large macrophages engulfing red cells and lymphocytes (hemophagocytosis). HE, 130x. B – vertebral bone marrow with focal finding of CD8+ lymphocytes rimming the adipocytes; the finding is highly suspect from infiltration by lymphoma, CD8 immunohistochemistry, 55x. C – mesentery with macrophages engulfing lymphocytes, HE, 73x. D – liver tissue showing marked dystrophic changes including steatosis and cholestasis of hepatocytes. HE, 70x. E – liver tissue without apparent lymphoma infiltration. CD3 immunohistochemistry, 12x. F – sample from skin and subcutis tissue without apparent lymphoma infiltration. HE, 2,5x
See this image and copyright information in PMC

References

    1. Jaffe ES, Gaulard P, Cerroni L. Subcutaneous panniculitis-like T-cell lymphoma. In: Swerdlow SH, Campo E, Harris NL, Jaffe ES, Pileri SA, Stein H, Thiele J, editors. WHO Classification of Tumours of Haematopoetic and Lymphoid Tissues (Revised 4th edition) Lyon: IARC; 2017. pp. 383–385.
    1. Kumar S, Krenacs L, Medeiros J, Elenitoba-Johnson KS, Greiner TC, Sorbara L, et al. Subcutaneous panniculitic T-cell lymphoma is a tumor of cytotoxic T lymphocytes. Hum Pathol. 1998;29(4):397–403. doi: 10.1016/S0046-8177(98)90122-8. - DOI - PubMed
    1. Willemze R, Jansen PM, Cerroni L, Berti E, Santucci M, Assaf C, et al. Subcutaneous panniculitis-like T-cell lymphoma: definition, classification, and prognostic factors: an EORTC cutaneous lymphoma group study of 83 cases. Blood. 2008;111(2):838–845. doi: 10.1182/blood-2007-04-087288. - DOI - PubMed
    1. Salhany KE, Macon WR, Choi JK, Elenitsas R, Lessin SR, Felgar RE, et al. Subcutaneous panniculitis-like T-cell lymphoma: clinicopathologic, immunophenotypic, and genotypic analysis of alpha/beta and gamma/delta subtypes. Am J Surg Pathol. 1998;22(7):881–893. doi: 10.1097/00000478-199807000-00010. - DOI - PubMed
    1. Lester L, Ewalt M, Warnke R, Kim J. Systemic panniculitis-like T-cell lymphoma with involvement of mesenteric fat and subcutis. J Cutan Pathol. 2015;42(1):46–49. doi: 10.1111/cup.12436. - DOI - PubMed

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