Affiliation:
1. Anemia Clinic, Children’s Hospital of Michigan, and Department of Pediatrics, Wayne University, Detroit, Mich.
Abstract
Abstract
A congenital familial chronic hemolytic anemia not conforming to the known types was observed in 3 members of a French-Canadian family.
The erythrocytes were normochromic, normocytic to macrocytic, and had normal osmotic and mechanical fragility. Spherocytosis. elliptocytosis and sickling were absent but a moderate degree of ovalocytosis was present in many of the patients’ red corpuscles. No acid hemolysis could be demonstrated. The survival of erythrocytes transfused from one of the patients to a normal recipient was significantly shortened, whereas normal erythrocytes transfused into 2 of the patients survived normally, indicating an intracorpuscular defect.
Clinically the condition was characterized by jaundice, hepatosplenomegaly, osseous changes, and a tendency toward the development of mongoloid facies. Typical hemolytic crises have been absent. Despite moderately severe anemia there has been remarkably little interference with normal childhood activity.
Publisher
American Society of Hematology
Subject
Cell Biology,Hematology,Immunology,Biochemistry
Cited by
33 articles.
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1. Hereditary Haemolytic Elliptocytosis;Acta Medica Scandinavica;2009-04-24
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