Two cases of Di Guglielmo syndrome (acute erythremic myelosis) are reported.
Case 1, a boy aged 12year and 2month, 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 mormoblasts 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 prednisolone, hematemesis and epistaxis occured frequently with persistent high fever until discharge on loth hospital day.
Case 2, a boy aged 3year and 11month, had intermittent high fever and watery diarrhea of about 15 days duration.
He had pale conjunctiva, moderate hepatosplenomegaly, anamia, 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 per-odic acrd-Scbiff reaction of the bone marrow smear was positive.
Whole blood transfusions and administrations of antibiotics were done without improvement, and he died on 6th hospital day.
A brief review of related literatures is given.
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