Abstract OBJECTIVE: To study the clinical features of hepatitis-associated aplastic anemia (HAAA) in children. METHODS: The clinical data of the children with newly diagnosed HAAA from January 2007 to December 2008 were respectively studied, including clinical manifestations, and blood routine, bone marrow examination, viral serology and immune function results as well as treatment and prognosis. RESULTS: A total of 8 children were confirmed as HAAA, accounting for 4.9% in children with aplastic anemia. There were 7 males and 1 female. The median age was 7.5 years (range 4.4 to 10.3 years) at diagnosis. They had negative serologic results and the causes of hepatitis could not be identified. The median interval from hepatitis occurrence to blood cell reduction was 6 weeks. Three cases were diagnosed as severe aplastic anemia and 5 cases as very severe aplastic anemia. Severe T cell immune disorders were found in all 8 cases. The percentage of Ts cells increased and the percentage of Th cells decreased significantly in the 8 children with HAAA. Four children survived after immune suppress treatment, three children died within one month after diagnosis and one child required own discharge without treatment. CONCLUSIONS: HAAA is more frequent in male school children. The children with HAAA have severe T cell immune disorders, with a higher early death rate. Immune suppress treatment is effective.[Chin J Contemp Pediatr, 2010, 12 (8):609-612]
[1]Baumelou E, Guiguet M, Mary JY. Epidemiology of aplastic anemia in France: a case-control study. I. Medical history and medication use. The French Cooperative Group for Epidemiological Study of Aplastic Anemia [J]. Blood, 1993, 81(6):1471-1478.
[4]Brown KE, Tisdale J, Barrett AJ, Dunbar CE, Young NS.Hepatitis-associated aplastic anemia [J]. N Engl J Med, 1997, 336(15): 1059-1064.
[5]Young NS, Issaragrisil S, Chieh CW, Takaku F. Aplastic anaemia in the Orient [J]. Br J Haematol, 1986, 62(1):1-6.
[6]Kirsch R, Yap J, Roberts EA, Cutz E.Clinicopathologic spectrum of massive and submassive hepatic necrosis in infants and children [J]. Human Pathol, 2009, 40(4): 516-526.
[7]Pongtanakul B, Das PK, Charpentier K, Dror Y. Outcome of children with aplastic anemia treated with immunosuppressive therapy [J]. Pediatr Blood Cancer, 2008, 50(1): 52-57.
[8]Savage WJ, DeRusso PA, Resar LM, Chen AR, Higman MA, Loeb DM, et al. Treatment of hepatitis-associated aplastic anemia with high-dose cyclophosphamide [J]. Pediatr Blood Cancer, 2007, 49(7): 947-951.
[9]Osugi Y, Yagasaki H, Sako M, Kosaka Y, Taga T, Ito T, et al.Antithymocyte globulin and cyclosporine for treatment of 44 children with hepatitis associated aplastic anemia [J]. Haematologica, 2007, 92(12): 1687-1690.
[10]Lu J, Basu A, Melenhorst JJ, Young NS, Brown KE. Analysis of T-cell repertoire in hepatitis-associated aplastic anemia [J]. Blood, 2004, 103(12): 4588-4593.