Modifying NHL-BFM-90 and HLH-2004 Protocols for a Child with SPLTCL and HLH; Prompt Initiation of Dexamethasone and Etoposide

Abstract

Subcutaneous panniculitis-like T-cell lymphoma (SPLTCL) which is similar to lobular panniculitis is a subtype of skin lymphoma that is characterized by pleomorphic T cells and benign macrophages. The simultaneous presence of hemophagocytic lymphohistiocytosis (HLH) is the most important and adverse prognostic factor in SPLTCL. SPLTCL is a rare disease with no well-established standard treatment. We report a child with SPLTCL and HLH, who were successfully treated with the modified NHL (non-Hodgkin lymphoma)-BFM(Berlin-Frankfurt-Münster)-90 and HLH-2004 protocols. Patient had persistent fever and subcutaneous masses. SPLT-CL with HLH was diagnosed by immunohistochemistry, radiology and laboratory results. SPLTCL with HLH has shown high mortality when treated with a combination of intensive anticancer drugs. Thus, we first administered dexamethasone and etoposide. After this, when we used the modified protocol of NHL-BFM-90 and HLH-2004, patient showed complete resolution of the subcutaneous masses and features of HLH, except for persistent hyperferritinemia. We tried etanercept to reduce high serum ferritin with some effects. In children with diagnosis of SPLTCL with HLH, initiation of immediate and appropriate treatment affects prognosis. Thus, prompt initiation of the agents that can simultaneously control underlying disease as well as secondary HLH could have lead to successful results.