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Di Gugliemo Syndrome (Acute Erythremic Myelosis)

Journal of the Korean Pediatric Society 1975;18(12):945-952.
Published online December 31, 1975.
Di Gugliemo Syndrome (Acute Erythremic Myelosis)
D. S. Kang1, S. M. Kim1, K. S. Lee1, H. J. Chang1, T. W. Kim1, C. I. Suh2, S. I. Kim3
1Dept, of Pediatrics, College of Medicine, Chung Nam University
2Dept, of Clinical Laboratory, College of Medicine, Chung Nam University
3Dept, of Clinical Laboratory College of Medicine, Seoul National University
Di Guglielmo 症候群 (Acute Erythremic Myelosis)
姜達三1, 金相滿1, 李建壽1, 張學鎭1, 金泰雲1, 徐廷翊2, 金相仁3
1忠南大學技 醫科大學 小兒科學敎室
2忠南大學技 醫科大學 臨床病理學敎室
3서울大學校 醫科大學 臨床症理學敎室
Abstract
Two cases of Di Guglielmo syndrome (acute erythremic myelosis) are reported. Case 1, a boy aged 12-year and 2-month, had diarrhea of about one month’s duration. There were marked emaciation, pale conjunctiva faint apical systolic murmur (on physical examination.) C.B.C. showed severe pancytopenia with some .normoblasts in the absence of immature myeloid cells on peripheral blood smear. Bone marrow was slightly hyperplastic with an increase in erythroid cells, some of which were megaloblastic, and large bizzare multinucleated erythroid cells were also noted. Despite of multiple whole blood transfusions and administrations of antibiotics and predn isolone, ' hematemesis and epistaxis occured frequently with persistent high fever until discharge on 10th hospital day. Case 2, a boy aged 3-year and 11-month, had intermittent high fever and watery diarrhea of about 15 days duration. He had pale conjunctiva, moderate hepatosplenomegaly, anemia, thrombocytopenia and mild leukocytosis. Many normoblasts were seen without immature myeloid cells on peripheral blood smear and the bone marrow findings were similar to the case 1. The periodic acid-Schiff reaction of the bone marrow smear was positive. Whole blood transfusions and administrations of antibiotics were done without improvem- ent, and he died on 6th hospital day. A brief review of related literatures is given.


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