Tissue oxygenation in patients with hemoglobinopathy H.
To evaluate the degree of tissue hypoxia in patients with hemoglobinopathy 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 are biphasic because of a combination of the rectangular hyperbolic curve of HbH and the normal sigmoid curve of HbA and are shifted toward the left (P50 3.66 +/- 0.33 kPa). Patients with iron deficiency anemia have right-shifted oxygen equilibrium curves (P50 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 patients (1.6 +/- 0.2 mmol/L) with a similar degree of anemia. Red cell indices vary between the two groups. In patients with HbH disease the mean corpuscular hemoglobin concentration was 268 +/- 17 g/L as compared with 294 +/- 18 g/L in iron deficiency anemia. These findings indicate that whole blood oxygen affinity is a reliable index of tissue oxygenation in patients with hemoglobinopathy H.
Papassotiriou
I
I
Hematology Laboratory, Aghia Sophia Children's Hospital, Athens, Greece.
Kanavakis
E
E
Stamoulakatou
A
A
Kattamis
C
C
D018798:Anemia, Iron-Deficiency / Q000097:blood / Q000378:metabolism*; D002851:Chromatography, High Pressure Liquid; D004912:Erythrocytes / Q000378:metabolism; D006441:Hemoglobin A / Q000378:metabolism*; D006447:Hemoglobin H / Q000032:analysis / Q000378:metabolism*; D006453:Hemoglobinopathies / Q000097:blood / Q000378:metabolism*; D010101:Oxygen Consumption; D010108:Oxyhemoglobins / Q000032:analysis* / Q000378:metabolism
D010108:Oxyhemoglobins; D006441:Hemoglobin A; D006447:Hemoglobin H