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November 1965

Familial Myeloproliferative Disease: A New Syndrome Closely Simulating Myelogenous Leukemia in Childhood

Author Affiliations

From the departments of pediatrics and biophysics of the University of Colorado School of Medicine and the Denver Children's Hospital. Former Resident in Pediatrics (Dr. Randall); Professor of Pediatrics (Dr. Githens); and Associate Professor of Pediatrics and Biophysics (Dr. Robinson), University of Colorado School of Medicine, and Clinical Pathologist and Hematologist, Denver Children's Hospital (Dr. Reiquam).

Am J Dis Child. 1965;110(5):479-500. doi:10.1001/archpedi.1965.02090030503001

THIS REPORT describes a myeloproliferative disease that has been observed in nine related children living in Eastern Colorado, Northern Texas, and Western Nebraska. The disease is characterized by an early onset of marked splenomegaly, hepatomegaly, anemia, thrombocytopenia, and a marked leukocytosis (up to 128,000). The bone marrow is extremely hyperplastic, involving particularly the granulocytic series. In the majority of cases, the cell distribution suggests chronic or subacute myelogenous leukemia. Most of these children were initially diagnosed as having leukemia; but the clinical course, the familial nature of the disease, and additional histologic data do not support this diagnosis. The occurrence in nine related children is particularly unusual. It is true that familial instances of leukemia have been described, but the histologic pattern and clinical course in them have been similar to those with the usual form.1

The various conditions in childhood which may be associated with a peripheral blood

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