SPTCL with Hemophagocytic Syndrome in a child

Vishal Gupta, Sudheer Arava, Sameer Bakhshi, Kanya Rani Vashisht, Rakesh Reddy, Somesh Gupta

Overview

Subcutaneous panniculitis-like T-cell lymphoma (SPTCL) characterized by subcutaneous infiltration of pleomorphic T-cells of the alpha/beta phenotype rarely affects children. Development of hemophagocytic syndrome (HPS) leads to a poor prognosis with this otherwise indolent lymphoma. We report a case of SPTCL in a 5-year-old child complicated by HPS treated successfully with combination chemotherapy.

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Subcutaneous Panniculitis-Like T-Cell Lymphoma with Hemophagocytic Syndrome in a Child.

Authors:
Dr. (Prof.) Somesh Gupta, MD, DNB, MNAMS
Dr. (Prof.) Somesh Gupta, MD, DNB, MNAMS
All India Institute of Medical Sciences
Professor
Dermatology and Venereology
New Delhi, Delhi | India
Dr. Kanya Rani Vashisht, MD
Dr. Kanya Rani Vashisht, MD
All India Institute of Medical Sciences (AIIMS)
MD Dermatology, Leprology & Venereology
Delhi | India

Pediatr Dermatol 2016 Mar-Apr;33(2):e72-6. Epub 2016 Jan 14.

Department of Dermatology and Venereology, All India Institute of Medical Sciences, New Delhi, India.

Subcutaneous panniculitis-like T-cell lymphoma (SPTCL) characterized by subcutaneous infiltration of pleomorphic T-cells of the α/β phenotype rarely affects children. Development of hemophagocytic syndrome (HPS) leads to a poor prognosis with this otherwise indolent lymphoma. We report a case of SPTCL in a 5-year-old child complicated by HPS treated successfully with combination chemotherapy. We discuss the potential pitfalls in reaching an early diagnosis and challenges in its management. Previously reported cases of SPTCL with HPS in children are briefly reviewed.

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Source
http://dx.doi.org/10.1111/pde.12776DOI Listing
January 2017
9 Reads
1 Citation
1.520 Impact Factor

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