Journal of Clinical and Experimental Hematopathology
Online ISSN : 1880-9952
Print ISSN : 1346-4280
ISSN-L : 1346-4280
Case Study
Subcutaneous Panniculitis-Like T-Cell Lymphoma (SPTCL) with Hemophagocytosis (HPS) : Successful Treatment Using High-Dose Chemotherapy (BFM-NHL & ALL-90) and Autologous Peripheral Blood Stem Cell Transplantation
Eiichi SakuraiTakashi SatohYashima-Abo AkikoChihaya MaesawaKanako TsunodaMikiya EndoToshihide AkasakaTomoyuki Masuda
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2013 Volume 53 Issue 2 Pages 135-140

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Abstract

Subcutaneous panniculitis-like T-cell lymphoma (SPTCL) is a rare form of non-Hodgkin lymphoma, in which lymphoma cells infiltrate preferentially into subcutaneous adipose tissue. Although various treatment trials for SPTCL have been attempted, no standardized therapy has been established. Here, we report a case of α/β+ T-cell-phenotype SPTCL (SPTCL-AB) with hemophagocytosis (HPS) in a 14-year-old girl, who presented with low-grade fever, general fatigue and chest swelling. Laboratory examinations revealed leukocytopenia, and bone marrow aspiration cytology showed HPS. The diagnosis of SPTCL-AB was made by biopsy on the basis of thickened subcutaneous tissue in the chest wall. Following high-dose chemotherapy (HDT) of BFM-NHL & ALL-90, autologous peripheral blood stem cell transplantation (auto-PBSCT) was performed. The patient responded to the treatment and has remained asymptomatic for 2 years. Our results suggest that a combination of HDT of BFM-NHL & ALL-90 and auto-SCT treatment is effective for SPTCL associated with HPS. [J Clin Exp Hematop 53(2) : 135-140, 2013]

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© 2013 by The Japanese Society for Lymphoreticular Tissue Research
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