Primary NK/T cell lymphoma with secondary hemophagocytic syndrome: A case report and literature review / 肿瘤

ABSTRACT

Objective: To explore the etiology and risk factors related to prognosis of primary natural killer (NK)/T cell lymphoma with secondary hemophagocytic syndrome (NK/T-LAHS) and review the advances in treatment of NK/T-LAHS. Methods: One case of primary NK/T cell lymphoma with secondary hemophagocytic syndrome was discussed in combination with a review of related literatures. Results: The patient was a 21-year old female who presented with high fever, hepato-splenomegaly, pancytopenia and jaundice. She was diagnosed with primary extranodal NK/T cell lymphoma (nasal type, ?B) with secondary hemophagocytic syndrome. A combined chemotherapy of etoposide, dexamethasone, L -asparaginase and high-dose methotrexate with vigorous supportive therapy was given. The patient's clinical condition was improved transiently after chemotherapy, but relapsed in a very short period and markedly deteriorated. Ultimately the patient refused treatment and took her own discharge againstadvice. Conclusion: Primary NK/T-LAHS is rare and has a rapid progression with poor prognosis. There are not effective treatments for NK/T-LAHS and novel therapeutic regimens should be investigated.