Hepatitis-Associated Aplastic Anemia. Kazuko KUDO and Seiji KOJIMA Department of Pediatrics, Nagoya University Graduate School of Medicine

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1 Hepatitis-Associated Aplastic Anemia Kazuko KUDO and Seiji KOJIMA Department of Pediatrics, Nagoya University Graduate School of Medicine Abstract We retrospectively analysed the clinical outcome of 85 children with hepatitis-associated aplastic anemia (HAA), who were diagnosed between April 1988 and March 2000 and registered by the Aplastic Anemia Committee of the Japanese Society of Pediatric Hematology. Twenty-three patients received bone marrow transplantation (BMT) and the other 60 patients were treated with immunosuppressive therapy (IST) as a first-line therapy. Twelve of the 16 nonresponders to IST received BMT as a second-line therapy. The results of viral studies were available in 70 patients, but the pathogenesis of HAA is still unclear. The association with HLA-DR was also examined in 48 HAA and 156 idiopathic aplastic anemia (IAA) patients. The increased presence of HLA-DR9 was more frequent in HAA (54.2%) than in normal populations (27.8%). This was not observed in patients with IAA. The actuarial survival rates at 8 years in patients who were diagnosed between 1988 and 1993 and after 1994 were 70.8 } 9.3% and %, respectively. They were similar between the patients with HAA and IAA. It was concluded that there was no statistical difference in the response rate to IST between the children with HAA and IAA. Further studies are required to clarify the etiology of the preceding hepatitis and the mechanism of bone marrow failure. Key words : hepatitis-associated aplastic anemia, bone marrow transplantation, immunosuppressive therapy, HLA-DR Reprint requests to Kazuko Kudo, Department of Pediatrics, Nagoya University Graduate School of Medicine, 65, Tsurumai-cho, Showa-ku, Nagoya, Japan

2 Table 2 Clinical characteristics of patients with hepatitis-associated aplastic anemia Table 1 Demographics and clinical characteristics of 85 patients with hepatitis-associated aplastic anemia

3 Fig. 1 Clinical course of patients with hepatitis-associated aplastic anemia Table 3 Serological and virological characterizations of hepatitis-associated aplastic anemia

4 Fig. 2 Kaplan-Meier estimate of actuarial survival in patients diagnosed before 1993(---) and after 1994( c) 1) Brown KE, Tisdale J, Barrett J, et al : Hepatitisassociated aplastic anemia. N Engl J Med 336: , ) Liang D, Lin K, Lin D, et al : Post-hepatitic aplastic anaemia in children in Taiwan, a hepatitis prevalent area. Br J Haematol 74 : , ) Kojima S, Matsuyama T, Kodera Y, et al : Circulating activated suppressor T lymphocytes in hepatitisassociated aplastic anaemia. Br J Haematol 71: , 1989

5 5) Ohara A, Kojima S, Okamura J, et al : Evolution of myelodysplastic syndrome and acute myelogenous leukemia in children with hepatitis-associated aplastic anaemia. Br J Haematol 116 : , ) Safadi R, Or R, Ilan Y, et al : Lack of known hepatitis virus in hepatitis-associated aplastic anemia and outcome after bone marrow transplantation. Bone Marrow Transplant 27 : , ) Iriyama M, Kimura H, Nishikawa K, et al : The prevalence of TT virus (TTV) infection and its relationship to hepatitis in children. Med Microbiol Immunol 188 : 83-89, ) Maciejewski J, Follmann, D, Nakamura R, et al : Increased frequency of HLA-DR2 in patients with paroxysmal nocturnal hemoglobunuria and the PNH/ aplastic anaemia syndrome. Blood 98: : ) Nakao S, Takamatsu H, Chuhjo T, et al : Identification of a specific HLA class II haplotype strongly associated with susceptibility to cyclosporinedependent aplastic anaemia. Blood 84: , 1994

Therapeutic Advances in Treatment of Aplastic Anemia. Seiji Kojima MD. PhD.

Therapeutic Advances in Treatment of Aplastic Anemia Seiji Kojima MD. PhD. Department of Pediatrics Nagoya University Graduate School of Medicine Chairman of the Severe Aplastic Anemia Working Party Asia-Pacific