－S 317 －
￭ S-509 ￭
A case of primary biliary cirrhosis presenting as chronic graft-versus-host disease after allogeneic stem cell transplantation
Departments of 1Internal Medicine, 2Laboratory Medicine, 3Pathology and Medical Research Center for Cancer Molecular Therapy, Dong-A University College of Medicine, Busan, Korea
*Hyun Hwa Yoon1, Kyung A Kwon1, Suee Lee1, Sung Yong Oh1, Hyuk-Chan Kwon1, Hyo-Jin Kim1, Kyeong-Hee Kim2, Jin-Yoeng Han2, Jin Sook Jeong3, Sung-Hyun Kim1
Graft-versus-host disease (GVHD) and primary biliary cirrhosis (PBC) are thought to have common immunopathologic and clinical features.
Furthermore, liver histology in both diseases is characterized by lymphocytic infiltration of the portal fields and destruction of small bile ducts.
Various autoantibodies including antimitochondrial antibody (AMA) had been reported in patients with chronic GVHD after allogeneic stem cell transplantation (SCT). Serum AMA are detected in more than 90% in PBC. Previous reported data on AMA in GVHD might be primarily false positives. We report a case of a PBC as chronic GVHD after allogeneic SCT. A 28-year-old female presented with arthralgia of her elbows, wrists and knees. Due to acute myeloid leukemia with t(6;9), she had received HLA-identical allogeneic SCT from her brother one and half years ago. On presentation, she had leukocytosis with eosinophilia and elevated alkaline phosphatase, and her autoimmune antibody profile showed positivity for AMA (positive with M2 specificity) with a high titer of antinuclear antibody. Liver histology showed compatible with PBC with mild disease activity. She has been treated with ursodeoxycholic acid and immunosuppressive therapy. To our knowledge, this is the first case in Korea of PBC showing typical symptoms, autoantibody and liver pathology associated with cGVHD after allogeneic SCT.
The prognosis and optimal treatment modalities need to be determined.
￭ S-510 ￭
Rituximab can control Evans’syndrome, manifested as chronic GVHD after allogeneic PBSCT, refractory to steroid and FK-506
Korea University Medical Center
*Suk-Young Lee, Se Ryeon Lee, Yong Park, Byung Soo Kim
Evans’ syndrome (ES) is a rare but hardly controllable manifestation of chronic GVHD. The role of rituximab in control of chronic GVHD has been reported in some studies. Here, we first show the effectiveness of rituximab for treatment of ES from chronic GVHD resistant to steroid and anti T lymphocyte agents. A 17-year-old woman diagnosed as acute myeloid leukemia (FAB M1) with karyotype of 46, XX, del (9) (p22) [4]/46, XX [16] had induction chemotherapy consisted of idarubicin and Ara-C and consolidation chemotherapy with a high dose of Ara-C 2 times. During the first complete remission (CR), allogenic peripheral blood hematopoietic stem cell transplantation (PBSCT) from an unrelated HLA matched, sex and ABO mismatched (A+←AB+) donor was attempted. Prophylaxis against GVHD consisted of tacrolimus and methotrexate. Plasmapheresis was performed for prevention of immune hemolysis. No acute GVHD was observed. Tacrolimus was maintained for prevention of chronic GVHD. On day 155 after transplantation, ES was diagnosed. From day 156, 250 mg of methylprednisone was injected for 4 days, and consecutive 125 mg and 62.5 mg of steroid was injected for 15 and 3 days, respectively. After finishing steroid injection, minor response for autoimmune hemolytic anemia (AIHA) and CR for idiopathic thrombocytopenic purpura was obtained. 50 mg of prednisone was maintained, and tapered to 40 mg. On day 210, platelet counts decreased again, and AIHA still sustained. ES in this case was thought to be refractory to steroid and anti T lymphocyte treatment, and rituximab was administrated. 4 weekly and consecutive 4 monthly 375 mg/m2 doses were scheduled. After the scheduled rituximab injection, Hb was 11.1 g/dL, and platelet was 201×103/μL. After day 565, all immunosuppressive agents, including steroid, were stopped and blood examination showed normal findings. Rituximab administration was introduced as a salvage treatment for ES in this case. Additional to the first successful trial of rituximab for control of ES from chronic GVHD after allogeneic PBSCT, this report might support the role of B lymphocytes in manifestation of chronic GVHD after allogeneic hematopoietic stem cell transplantation.
