| Objective We report the two case of EBV-PTLD after allogeneic hematopoietic stem cell transplantation (Allo-HSCT), who were managed with Rituximab. Methods We retrospectively analyzed 186 cases of PTLD who received allo-HSCT from 1996 to 2008. The patients were divided into two groups: ATG-containing regimen group (21 patients) and non-ATG-containing regimen group (165 Patients). The two cases of PTLD were reported with literature review. Results Two cases out of 186 patients developed PTLD (1.1%). PTLD occurred in two patients in ATG-containing regimen group (10.5%), however, none of patients in non-ATG-containing regimen group developed PTLD (0%). Case1: A five-year-old girl with CML underwent a major ABO-incompatible HLA-identical unrelated cord blood transplantation (U-CBT) in January 2003. She was prepared with ATG-based conditioning regimen. The post-transplant course was complicated by pure red cell aplasia. She presented on day +75 with fever, followed by rapid enlargement of tonsillars, and a cluster of lymph nodes in the bilateral 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-PLTD and PRCA. Case2: An eleven-year-old boy with thalassemia-major underwent HLA-identical related peripheral blood stem cell transplantation in December, 2006. He was prepared with ATG-based conditioning regimen. The clinical course was complicated by capillary leak syndrome day +2 post-transplantation, which was successfully treated with Methylpredisonlone. He presented on day +45 with fever, followed by rapid enlargement of tonsillars, and a cluster of lymph nodes in the bilateral cervical regions. A cervical lymph node biopsy revealed the histopathologic findings consistent with B-cell PTLD. He developed edema, progressive shortness of breath, hypoxemia whit high fever three hours after the infusion of Rituximab, which is consistent with the diagnosis of Rituximab-induced capillary leak syndrome. The treatment was withdrawn because of the potential risk of acute respiratory failure caused by Rituximab although he responded rapidly to Rituximab and achieved partial remission. Treatment was restarted with a half dose of Rituximab since the disease relapsed and his clinical condition was getting worse, but he finally died of respiratory failure which was aggravated by Rituximab. Conclusion EBV-PLTD may occur in increasing frequencies due to the increasing numbers of transplant recipients, transplant physician should be especially alert to this life-threatening complication. ATG is a high-risk factor of developing PTLD. Rituximab alone may be an effective therapeutic option for patients who develop EBV-PTLD, but it should be cautious for patients with a history of lung dysfunction because of the potential risk of acute respiratory failure induced or aggravated by Rituximab. |
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