TISSUE OXYGENATION IN PATIENTS WITH HEMOGLOBINOPATHY-H

To evaluate the degree of tissue hypoxia in patients with hemoglobinop athy H disease, whole blood oxygen affinity was estimated and analyzed in 33 patients. Twenty patients with Iron deficiency anemia, matched for degree of anemia, served as controls. The results were as follows: Whole blood oxygen equilibrium curves of patients with HbH disease ar e Diphasic because of a combination of the rectangular hyperbolic curv e of HbH and the normal sigmoid curve of HbA and are shifted toward th e left (P-50 3.66 +/- 0.33 kPa). Patients with iron deficiency anemia have right-shifted oxygen equilibrium curves (P-50 4.02 +/- 0.13 kPa) compared with normal. Oxygen release to the tissues in HbH disease is decreased (1.4 +/- 0.3 mmol/L) as compared with iron-deficient patient s (1.6 +/- 0.2 mmol/L) with a similar degree of anemia. Red cell indic es vary between the two groups. In patients with HbH disease the mean corpuscular hemoglobin concentration was 268 +/- 17 g/L as compared wi th 294 +/- 18 g/L in iron deficiency anemia. These findings indicate t hat whole blood oxygen affinity is a reliable index of tissue oxygenat ion in patients with hemoglobinopathy H.