Abstracts - 2022 - Haemophilia

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thalassemia microcytic hypochromic anemia :: Article Creator

HEART DISEASE IN THALASSEMIA HETEROZYGOTES WITH SICKLE CELL ANEMIA

The purpose of this study was to ascertain whether thalassemia heterozygotes (α thal) who have sickle cell anemia (SCA) suffer fewer cardiac effects of their SCA due to an increased oxygen carrying capacity or decreased sickling. Echocardiograms and graded, maximal exercise tests were performed in 22 subjects withα thal and SCA, and in 22 age and sex matched controls (C) with SCA alone. The patients ranged in age from 8-32 years. None were ill within two weeks of study. No significant differences were found for any of the following measurements: age (α thal 19.0 years, C 18.5); hemoglobin (α thal 8.8 gm%, C 8.2); hemoglobin F (α thal 6.6%, C 7.8); body surface area (α thal 1.43 M2, C 1.45) heart rate (α thal 78.6, C 80.3); left ventricular (LV) dimension (α thal 5.38 cm., C 5.42); LV wall thickness (α thal 0.91 cm., C 0.92); right ventricular (RV) dimension (α thal 2.26 cm., C2.42); left atrial dimension (α thal 3.70 cm., C 3.91); shortening fraction (α thal 34.8%, C 36.5); and work capacity (α thal 7.3 kg-m/min/kg, C 8.4. Exercise induced S-T depression was noted in 8/44 (18%) of subjects. The "α thal group was more often affected 6/8 (NS). α thal was not protective in heterozygotes with SCA. Abnormalities attributable to increased viscosity at capillary level persist in α thal heterozygotes with SCA. α thal homozygotes would thus be an ideal group to examine these effects independent of the manifestations of severe anemia, since they tend to have higher hemoglobins than sickle cell patients without α thal.





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