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Erythraemic Myelosis (di Guglielmo) in an Infant
Author(s) -
WEGELIUS RUTH,
PELTONEN TUOMAS
Publication year - 1954
Publication title -
acta pædiatrica
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.772
H-Index - 115
eISSN - 1651-2227
pISSN - 0803-5253
DOI - 10.1111/j.1651-2227.1954.tb04028.x
Subject(s) - reticulocytosis , medicine , splenectomy , erythropoiesis , spleen , physiology , peripheral blood , pediatrics , reticulocyte , blood transfusion , anemia , surgery , immunology , biochemistry , chemistry , messenger rna , gene
Summary An infant of 2 months exhibited aneanaemia, combined with a hyperplastic reaction of the erythropoietic organs. Nucleated red blood cells were found in the peripheral blood but no reticulocytosis. The spleen was enlarged. In spite of the hyperplasia of the erythropoietic organs no sign of increased erythropoiesis could ever be found. The nucleated red cells showed signs of disturbance in the normal course of maturation, mainly evidenced by a persistent basophilia of the cytoplasm and by severe malformations of the nucleus in a later stage of maturation. No antianaemic agents had any effect on the anaemia and the infant was kept alive by repeated, continuous blood transfusions. He was also given a course of ACTH with apparent good effect. Splenectomy was performed when the child was in good condition after a course of ACTH and continuous transfusion. After this the child was temporarily in comparatively good health but in no better condition than when receiving continuous blood transfusions. The illness lasted for one year and five months after the child was admitted to hospital the first time, and ended with his death.