Summary:Hemophagocytic syndrome (HPS) associated with T cell lymphoma is well known, but an association with B cell lymphoma is rare. We report a patient who developed subcutaneous non-Hodgkin's lymphoma (NHL) of B cell origin. Autologous peripheral blood stem cell transplantation (PBSCT) was undertaken in her second complete remission (CR), but HPS appeared 5 months after transplantation. The patient deteriorated rapidly and died of metabolic acidosis. Necropsy revealed that she had relapsed B cell NHL involving liver, spleen and bone marrow. B cell NHL after PBSCT, associated with HPS as an initial sign, is rare. We report this case and review the literature. Keywords: subcutaneous B cell lymphoma; PBSCT; hemophagocytic syndrome Although T cell lymphoma-associated hemophagocytic syndrome (HPS) is frequently observed, B cell lymphomaassociated HPS is rare. We report a case of B cell nonHodgkin's lymphoma (NHL) with HPS after peripheral blood stem cell transplantation (PBSCT).
Case reportA 43-year-old previously healthy female noticed two skin tumors (1 cm and 1.5 cm in diameter) underneath the right breast in January 1994. Histological diagnosis of the resected tumors was diffuse large cell NHL, according to the Working Formulation classification, which appeared to grow from the subcutaneous tissue (Figure 1a). Immunohistochemical study revealed that the tumor cells were derived from B cells. She was then referred to our hospital for further examination and treatment. No nodal or extranodal involvement was found apart from the original subcutaneous tumors, and clinical stage of I EA was defined. She achieved complete remission (CR) after six cycles of CHOP (cyclophosphamide, doxorubicin, vincristine and prednisolone) chemotherapy. Two months later, subcutane- ous tumors developed on her face, breast and thighs. Recurrent NHL was diagnosed, based on histological study of the tumors. On surface marker analysis, the lymphoma cells were positive for CD10, CD19, CD20 and HLA-DR. Southern blot analysis showed rearrangement of the immunoglobulin heavy chain gene (IgH) but not the TCR gene. A second CR was achieved with one course of salvage chemotherapy comprising carboplatin, cytosine arabinoside, etoposide, mitoxantrone, and prednisolone. In July 1995, autologous PBSCT was carried out with 2.2 × 10 6 /kg of CD34-positive cells, following a preparatory regimen