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A Case of Congenital Dyserythropoietic Anemia, Type II

Journal of the Korean Pediatric Society 1994;37(1):99-103.
Published online January 15, 1994.
A Case of Congenital Dyserythropoietic Anemia, Type II
Won Kyung Yang1, Jung Wan Yoo1, Hyung Goo Cho1, Dong Chul Park1, In Sung Lee2, Won Yong Lee3
1Department of Pediatrics, College of Medicine, Kon-Kuk University, Chung-Ju, Korea
2Department of Pathology, College of Medicine, Kon-Kuk University, Chung-Ju, Korea
3Department of Microbiology,Yonsei University College of Medicine, Seoul, Korea
제 2형 선천성 적혈구 이형성 빈혈 1례
양원경1, 유정완1, 조형구1, 박동철1, 이인성2, 이원영3
1건국대학교 의과대학 소아과학교실
2건국대학교 의과대학 임상병리학교실
3연세대학교 의과대학 미생물학교실
Congenital dyserythropoietic anemia Type II (herditary erythroblatic multinuclearity with positive acidfied serum test;HEMPAS) is characterized by binuclearity, multinuclearity, pluripolar mitoses, karyorrhexis of normoblasts, and the presence of abnormla antigens on the red cells. We experienced a case of HEMPAS in a 2 month old girl patient who had an intermittent fever, abdominal distention with palpable liver & spleen, and generalized jaundice. The blood analysis revealed anemia, and thrombocytopenia. Peripheral blood smear showed an averge of 6 mature normoblast per 100 leukocyte count. The red cells showed moderate anisocytosis, poikilocytosis, irregularly crenated, cntracted cells and occasional spherocytes. The leukocytes showed relative lymphocytosis, and there were occasional villous lymphocytes. The marrow smear showed abnormality in erythroid series. About ten percent of the erythroblasts showed 2~7 nuclei or lobulated nuclei. The mitotic forms of the erythroid precursors were also increased in frequency. The M:E ratio was 1:3.2. Blood culture on this patient showed a pure growth of Coxiella burnetti. Medical treatment with Doxycycline and Rifampin was performed. After treatment, she became afebrile showing improved general condition with decreased size of liver & spleen. In spite of clinical improvement, she died suddenly 3 weeks after initial treatment.
Key Words: Congenital dyserythropoietic anemia, Coxiella burnetti

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