Treatment of Epstein-Barr virus--associated lymphoproliferative disorder (EBV-PTLD) and pure red cell aplasia (PRCA) with Rituximab following unrelated cord blood transplantation: a case report and literature review.

ABSTRACT

OBJECTIVE: We report the first case of EBV-PTLD and pure red cell aplasia (PRCA) after an unrelated cord blood transplant in China, who was successfully treated with Rituximab. METHODS: Case report and literature review. RESULTS: A 5-year-old girl with CML underwent a major ABO-incompatible HLA-identical unrelated cord blood transplantation (U-CBT) in January 2003. The post transplant course was complicated by PRCA. She presented on day +75 with fever, followed by rapid enlargement of tonsils, and a cluster of lymph nodes in the cervical and submandibular regions. A cervical lymph node biopsy revealed the histopathologic findings consistent with PTLD. The immunoblasts were shown to contain EBV viral genomic DNA by PCR, and immunocytochemistry study for the latent membrane protein 1 (LMP-1) and in situ hybridization for Epstein-Barr encoded RNAs1 (EBER1) were both positive. She responded rapidly to Rituximab and achieved complete resolution of clinical findings and symptoms of both EBV-PTLD and PRCA. CONCLUSION: EBV-PTLD may occur with increasing frequency due to the increasing numbers of transplant recipients, transplant physician should be aware of this life-threatening complication. Rituximab alone may be an effective therapeutic strategy for patients who develop EBV-PTLD and PRCA after U-CBT.