Case report of unrelated cord blood transplantation for the treatment of recurrent refractory Epstein-Barr virus-associated hemophagocytic lymphohistiocytosis with intestinal perforation and literature review / 中华实用儿科临床杂志

ABSTRACT

Objective:To examine the clinical experience and efficacy of unrelated cord blood transplantation (UCBT) in the treatment of recurrent refractory Epstein-Barr virus-associated hemophagocytic lymphohistiocytosis (EBV-HLH) in children.Methods:The clinical data of a patient with recurrent refractory EBV-HLH and intestinal perforation who was treated by UCBT in Department of Pediatrics, the First Affiliated Hospital of Zhengzhou University in September 2015 and finally cured were retrospectively analyzed.Meanwhile, literature was reviewed.Results:The patient, male, 1 year and 6 months, was admitted to the hospital with " fever for 15 days, rash for 9 days" as the main complaint, mainly manifested as high fever, large liver, spleen, lymph nodes, rapidly progressing pancytopenia, liver function damage, phagocytic blood cells on bone marrow smear, diagnosed as EBV-HLH in September 2015.The patient received chemotherapy according to the HLH-2004 protocol developed by the International Association of Cell Societies.During the treatment, he suffered two recurrence during the maintenance period, and a second-line rescue treatment was adopted, namely, " Pegaspargase, Doxorubicin liposome, Etoposide and Methylprednisolone" (L-DEP regimen) chemotherapy.The complete relief of diagnostic indexes for hemophagocytic lymphohistiocytosis was evaluated after chemotherapy.The patient developed sudden intestinal perforation and underwent emergency surgical surgery, enteroenterostomy.After the condition was stabilized, the patient was pretreated with the " Fludarabine+ Busulfan+ Cyclophosphamide" (Flu+ BU+ CY) therapy and then treated with UCBT, with intravenous nutritional support provided during the entire process.Neutrophil and platelet implantation was implemented on day 13 and day 35 after transplantation, respectively.The chimeric rate was 100%, and the implantation was a success.Hepatic veno-occlusive disease, fungal pneumonia and skin graft-versus-host disease (GVHD) Ⅱ occurred on the 15 th day, 22 nd day and 26 th day after transplantation, respectively.The corresponding symptoms improved after treatment.On day 49 after transplantation, phase Ⅱ " enterostomy fistula" was performed.The patient was followed up to 70 months after transplantation, and generally in good condition.His symptoms relieved, and no chronic GVHD and other comorbidities occurred. Conclusions:Allogeneic hematopoietic stem cell transplantation is the only possible effective means of treating relapsed refractory EBV-HLH in children.In the absence of a suitable sibling or unrelated donor, unrelated cord blood stem cells can be used as a graft source.Enterostomy after intestinal perforation is not contraindicated for transplantation.