PEDIATRICS Vol. 35 No. 6 June 1965, pp. 906-915
This Article
Right arrow Full Text (PDF)
Right arrow P3Rs: Submit a response
Right arrow Alert me when this article is cited
Right arrow Alert me when P3Rs are posted
Right arrow Alert me if a correction is posted
Services
Right arrow E-mail this article to a friend
Right arrow Similar articles in this journal
Right arrow Similar articles in PubMed
Right arrow Alert me to new issues of the journal
Right arrow Add to My File Cabinet
Right arrow Download to citation manager
Right arrow reprints & permissions
Citing Articles
Right arrow Citing Articles via CrossRef
Right arrow Citing Articles via Google Scholar
Google Scholar
Right arrow Articles by Miller, G.
Right arrow Articles by MacWhinney, J. B.
Right arrow Search for Related Content
PubMed
Right arrow PubMed Citation
Right arrow Articles by Miller, G.
Right arrow Articles by MacWhinney, J. B.

A NEW CONGENITAL HEMOLYTIC ANEMIA WITH DEFORMED ERYTHROCYTES (? "STOMATOCYTES") AND REMARKABLE SUSCEPTIBILITY OF ERYTHROCYTES TO COLD HEMOLYSIS IN VITRO

I. Clinical and Hematologic Studies

Gerald Miller M.D.1, Philip L. Townes Ph.D., M.D.1, and James B. MacWhinney M.D.1

1 Departments of Pediatrics and Anatomy, University of Rochester School of Medicine and Dentistry

A new variant of congenital hemolytic anemia is described. The principal clinical findings are moderately severe hemolytic anemia, jaundice, and splenomegaly; congenital ptosis of an eyelid may possibly be an associated anomaly. Splenectomy resulted in clinical improvement but did not completely arrest abnormal hemolysis nor alter red cell abnormalities. Laboratory data are presented which characterize the condition; the principal abnormalities detected thus far are: (1) abnormal morphology of the erythrocytes ("stomatocytes"); ) increased osmotic fragility and autohemolysis; (3) storage instability greater at 5°C than at 37°C; (4) decreased concentration of reduced glutathione despite normal G-6-P-D and G-6-P-D coupled glutathione reductase activities. The disorder is compared with hereditary spherocytosis and hereditary stomatocytosis; similarities to and differences from these disorders are reviewed.

Submitted on August 24, 1964
Accepted on November 23, 1964