Abstract:

Objective To explore the clinical characteristics and effective strategies of extranodal natural killer/T-cell lymphoma (ENKTL)-related hemophagocytic sydrome. Methods The clinical data and therapeutic strategies of a case of ENKTL-related hemophagocytic sydrome admitted to the Department of Hematology of the Second Affiliated Hospital of Anhui Medical University were retrospectively analyzed. Results A 17-year-old female patient with hemophagocytic sydrome onset was diagnosed with ENKTL by skin biopsy. The patient was also complicatedly infected with Epstein-Barr (EB) virus. The patient's body temperature, blood routine, fibrin, triglyceride, ferritin, bone marrow hemophagy and other hemophagocytic sydrome indicators were completely normal after 4 courses of treatment with programmed death 1(PD1) monoclonal antibody combined with pegaspargase, gemcitabine, and oxaliplatin (P-GEMOX) regimen, ENKTL had partial remission through efficacy evaluation, and EB virus(EBV) DNA quantitatively decreased significantly. The haploid allogeneic hematopoietic stem cell transplantation was performed subsequently, EBV was negative, and the disease continued to be in remission state. Conclusion Early diagnosis on ENKTL-related hemophagocytic sydrome with definite active EB viral infection and appropriate immunochemotherapy plus haploid allogeneic hematopoietic stem cell transplantation are critical for improved prognosis. The successful diagnosis and treatment of the patient provides certain reference for clinical practice.

Key words: lymphohistiocytosis, hemophagocytic, lymphoma, extranodal NK-T-cell, herpesvirus 4, human, antibodies, monoclonal, hematopoietic stem cell transplantation