All issues > Volume 18(12); 1975

Case Report

J Korean Pediatr Soc. 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

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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