Clinical study of patients with nasal NK/T cell lymphoma associated hemophagocytic syndrome

Objective To study clinical features, treatment and prognosis of nasal NK/T cell lymphoma associated Hemophagocytic Syndrome (HPS). Methods Retrospectively analysis method was used to analyze the clinical data of 3 patients with nasal NK/T cell lymphoma associated HPS. Results 3 patients with nasal NK/T cell lymphoma fulfilled the criteria of HPS. All patients had adverse prognostic factors of lymphoma. I patient developed HPS as the main primary manifestations of underlying lymphoma, the other 2 patients developed HPS during lymphoma progression. In three cases, bone marrow was infiltrated with lymphoma cells. When HPS occurred, the disease progressed rapidly. The most obvious clinical features were fever, pancytopenia, hypofibrinogenemia, hyperferritinemia, and hemophagocytosis in bone marrow. After being treated according to the HLH-2004 combined with chemotherapy, all patients showed a clinical response, but with the progression of lymphoma, HPS quickly relapsed, and all patients died of severe hepatic dysfunction, coagulopathy, or DIC. Conclusion Nasal NK/T lymphoma associated HPS is an invariably fatal disease with poor prognosis, typically occurring at advanced stage or the terminal phase of the disease. HLH- 2004 based protocol in combination with chemotherapy is helpful for nasal NK/T cell lymphoma associated HPS, which may delay disease progression and provid opportunities for the treatment of primary disease.