Key words: pure red cell anemia, allogeneic bone marrow transplantation.

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1 Complete Recovery of Hemopoiesis Following Bone Marrow Transplantation in a Patient with Unresponsive Congenital Pure Red Cell Anemia Masami SHIMADA, Hideo MUGISHIMA, Mitsuhiko HARA, Masahiro MISAWA, Hideo TAKAHASHI, Takashi SUZUKI, Masataka ICHIKAWA, Kokichi KAWADA, Takahito FUJISAWA, Masahiko OKUNI and Shigemi OSHIDA* Department of Pediatrics and *Department of Legal Medicine, Nihon University School of Medicine Abstract Bone marrow transplantation (BMT) was carried out on a 1-year-old boy with congenital pure red cell anemia who did not respond to corticosteroids, cyclophosphamide, and antilymphocyte globulin. Peripheral blood examination at diagnosis showed WBC 6,900/mm3, RBC 236 x 104/mm3, Hb 8.3 g/dl, Plat. 62 x 104/mm3, Reticulo. 0 yoo, and blood type was group 0 Rh (D)+. He had an HLA identical 4-year-old brother whose blood type was group A Rh (D) +. After he received cyclophosphamide (50 mg/kg for 4 days) and busulfan (3 mg/kg for 4 days) followed by 350 rads of total body irradiation, nucleated marrow cells (2.8 x 108/kg) were transfused on January 26th, Short-term MTX and CSA was given to prevent acute GVHD. No evidence of severe complication has developed after transplantation. The recovery of WBC over 1,000/mm3, neutrophil over 500/mm3, and platelet over 5 x 104/mm3 was day 19, 28, and 26, respectively. Bone marrow examination on day 58 showed the marked evidence of erythropoiesis and engraftment was confirmed by the blood type on day 120. He has been completely healthy and hematologically normal for more than 2 years. This is the first successful case of allogeneic transplant for congenital pure red cell anemia in Japan.

2 Key words: pure red cell anemia, allogeneic bone marrow transplantation.

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6 4) Marmont AM: Congenital hypoplastic anemia refractory of corticosteroids but responding to cyclophosphamide and antilymphocytic globulin. Acta Haematol 60: 90-99, ) Champlin R, Winston Ho, Gale RP: Antithymocyte globulin treatment in patients with aplastic anemia. N Engl J Med 308: , ) August CS, King E, Githens JH, et al: Establishment of erythropoiesis following bone marrow transplantation in a patient with congenital hypoplastic anemia. Blood 48: , ) Nakayama K, Nakagawa T : Prevalence and clinical states of aplastic anemia among children in Japan. Apla,stic anemia, Univ of Tokyo Press Tokyo 1978, 207 9) Diamond LK, Wang WC, Alter BP: Congenital hypoplastic anemia. Adv Pediatr 22: , 1976

7 382 II. Report of a second patient with an antibody to erythroblast nuclei and remission after immunosuppressive therapy. Blood 34: 1-13, ) Freedman MH, Amato D, Saunders EF : Erythroid colony growth in congenital hypoplastic anemia. J Clin Invest 57: , ) Hoffman R, Zanjani ED, Vila J, et al: Diamond.- Blackfan syndrome: Lymphocyte-mediated suppression of erythropoiesis. Science 193: , ) Nathan DG, Hillman DG, Chess L, et al: Normal erythropoietic helper T cell in congenital hypoplastic (Diamond-Blackfan) anemia. N Engl J Med 298: , ) Triondo A, Garijo J, Baro J, et al: Complete recovery of hemopoiesis following bone marrow transplant in a patient with unresponsive congenital hypoplastic anemia. Blood 64: , ) Lenarsky C, Weinberg K, Guinan E, et al: Bone marrow transplantation for constitutional pure red cell aplasia. Blood 71: , 1988

8 The Bulletin of Meiji University of Integrative Medicine API II 61 ASO X 11 7 X-4 6 X m 5 X-2 4 X 3 9 X 11 7 API 0.84 ASO X 1 1 MR-angio

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