Subcutaneous panniculitis-like T-cell lymphoma with hemophagocytic syndrome: a clinical and pathologic study of 6 cases

Subcutaneous panniculitis-like T-cell lymphoma (SPTCL) is a rare cutaneous lymphoma characterized by neoplastic T cell infiltration of the subcutaneous tissue. Approximately 15% of patients with SPTCL show hemophagocytic syndrome (HPS). This study aimed to analyze patients diagnosed with SPTCL and HPS. We retrospectively reviewed 6 cases of SPTCL with HPS, and described the clinicopathologic features, immunophenotypic findings, treatment, and prognosis. All patients initially presented with HPS. Mean patient age was 24 years (range: 17-31 years), with a male predominance. Histologically, biopsies from all patients showed infiltrates of small-to-medium lymphoid cells mimicking panniculitis. All cases were positive for CD2, CD3, CD7, CD8, TIA1, Granzyme-B, and TCRΒF1, and negative for CD4, CD20, CD56, CD30, and Epstein-Barr virus in situ hybridization. TCR gene rearrangement was clonal in 2 of 2 analyzed cases. One patient refused treatment and died after 2 weeks, and the remaining 5 patients received chemotherapy based on the cyclophosphamide, doxorubicin, vincristine, and prednisolone regimen. In addition, 3 patients received autologous or allogeneic hematopoietic stem cell transplantation (HSCT). Clinical follow-up data was available for 5 of 5 treated patients. Two patients died with disease and 3 patients were alive with no evidence of disease during the follow-up period. SPTCL with HPS is very rare, and patients can present with HPS as the first symptom. Some patients show rapid disease progression, and the application of high dosage chemotherapy combined with autologous or allogeneic HSCT should be considered.