Subcutaneous Panniculitis-Like T-Cell Lymphoma with Hemophagocytic Syndrome in a Child: A Successful Treatment with the BFM-NHL-90 Protocol

Abstract

Subcutaneous panniculitis-like T-cell lymphoma (SPTCL) is a distinct cutaneous lymphoma subtype that is characterized by pleomorphic T-cell infiltration of the subcutaneous tissue. SPTCL is usually associated with indolent clinical course. However, it can be complicated by hemophagocytic syndrome (HPS), which leads to worse prognosis. Childhood SPTCL is rare and there is no standardized treatment regimen of SPTCL with HPS. Here we report a pediatric case of SPTCL with HPS who responded favorably with multi-agent chemotherapy of the BFM (Berlin‐Frankfurt‐Münster)-NHL (non-Hodgkin lymphoma)-90 protocol.

Keywords

• subcutaneous panniculitis-like t-cell lymphoma
• hemophagocytic syndrome
• bfm-nhl-90