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Blood, 1966, Vol. 28, No. 6, pp. 830-839.
© 1966 American Society of Hematology, Inc.


Further Cases of Hb Q-H Disease (Hb Q-agr Thalassemia)

LIE-INJO LUAN ENG 1, R. P. PILLAY 1, and V. THURAISINGHAM 1

1 G. W. Hooper Foundation and the Hematology Unit, Department of Medicine, University of California Medical Center, San Francisco, Calif.; the General Hospital, Kuala Lumpur, Malaysia; and the General Hospital, Penang, Malaysia.

Case reports of four patients, all Chinese, with Hb Q-H disease—also called Hb Q-agr thalassemia—are presented. Three were siblings. Symptoms of chronic hemolytic anemia with jaundice and hepatosplenomegaly were present in all four subjects. The red blood cells were microcytic. Slight hypochromia was present in three of the cases. Poikilo- and anisocytosis with target cells and small intraerythrocytic crystals were found in the blood. Starch-gel electrophoresis revealed the presence of a large amount of Hb Q, a small amount of Hb H, and a minor slow-moving hemoglobin component with a mobility as much behind Hb A2 as Hb Q was behind Hb A. A small amount of Hb "Bart’s" was probably also present. The minor slow-moving component was thought to represent Hb agr2Qdgr2A2 or Hb Q2. Hb A and Hb A2 were not seen except after recent blood transfusion. Study of hemoglobin polypeptide chains showed the presence of normal betaA-chains and abnormal agrQ-chains, without demonstrable agrA-chains in the first three patients. In Patient #4 normal agrA-chains were demonstrable only after recent blood transfusion. The mother of the three siblings was heterozygous for Hb A; the father had agr-thalassemia trait.

Submitted on September 7, 1965
Accepted on April 23, 1966


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J. Clin. Pathol.Home page
K F S Leung, E S K Ma, A Y Y Chan, and L C Chan
Clinical phenotype of haemoglobin Q-H disease
J. Clin. Pathol., January 1, 2004; 57(1): 81 - 82.
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