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KMID : 0367419940370010099
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Journal of Korean Pediatric Society
1994 Volume.37 No. 1 p.99 ~ p.103
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A Case of Congenital Dyserythropoietic Anemia, Type II
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양원경
유정완/조형구/박동철/이인성/이원영
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Abstract
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Congenital dyserythropoietic anemia Type II (herditary erythroblatic multinuclearity with positive acidified serum test; HEMPAS) is characterized by binuclearity, multinuclearity, pluripolar mitoses, karyorrhexis of normoblasts, and the presence
of
abnormal
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, contracted 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.
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