Subcutaneous Panniculitislike T-cell Lymphoma With Hemophagocytosis: Complete Remission With BFM-90 Protocol

• DOI: 10.1097/mph.0b013e31817588e8
• Publication Date: 2009-03-05T19:17:05Z
• Authors: Kunjahari Medhi; Rajive Kumar; Arvind Rishi; Lalit Kumar; Sameer Bakhshi
• Keywords: Hemophagocytosis; Regimen; T-Cell Lymphoma

Subcutaneous panniculitislike T-cell lymphoma (SPTCL) is an uncommon type of cutaneous lymphoma. In many cases, SPTCL accompanied by hemophagocytic syndrome (HPS), resulting in prominent systemic symptoms. The natural history, optimal treatment strategy, and prognostic factors associated with this malignancy are not well defined. We report 11-year-old boy HPS who was initially treated conventional cyclophosphamide, doxorubicin, vincristine, prednisone chemotherapy, but progressed later on therapy. Subsequently, the child multiagent combination chemotherapy as per BFM-90 protocol achieved complete remission, has remained so for 3 years. This suggests value particular regimen patients HPS.