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

ABSTRACT

Subcutaneous panniculitis-like T-cell lymphoma (SPTCL) characterized by subcutaneous infiltration of pleomorphic T-cells of the α/ß 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.