Hemoglobin H disease in the Al-Qatif Region of Saudi Arabia

The Al-Qatif region in the Eastern province of Saudi Arabia has the highest prevalence of a-thalassemia genes in the Kingdom. Hemoglobin H (Hb H) disease, however, has been rarely reported. We decided therefore to verify the rarity of the disease, and characterize the presenting features in cases identified.

All patients seen in Qatif Central Hospital between September 1988 and November 1990 with low red cell indices were screened for Hb H disease, and those found positive had clinical data compiled from their hospital records and analyzed.

Thirty-nine cases of Hb H were diagnosed. The mean age of the patients was 18 years. The mean hemoglobin was 13.5 g/dl for neonates and 7.6 g/dl for the others. The mean Hb Bart's level was 27.5% in neonates and the mean Hb H level in others was 11.1%. In addition to low red cell indices, all patients had a high red cell distribution width (RDW) mean of 25.6%. The main clinical signs were jaundice and hepatosplenomegaly. Concurrent glucose-6-phosphate dehydrogenase (G6PD) deficiency was seen in 28.2% of patients.

Hemoglobin H disease is not uncommon in the Al-Qatif region of Saudi Arabia. The red cell indices may mimic iron deficiency, which should be excluded by the presence of jaundice and organomegaly. The condition often co-exists with G6PD deficiency.