Alpha (α)-thalassemia represents a group of recessively inherited hemoglobin disorders marked by deficient or absent synthesis of 1 to all 4 of the α-globin genes. Inactivation of 3 α-globin genes--either by deletional or nondeletional mutations--results in hemoglobin H (Hb H) disease. Patients with Hb H disease produce hemoglobin composed of all beta chains and have moderate to severe hemolyti...

We report the case of an Italian infant girl hand, in the Askenazy carriers of Hb Hafrom Polesine (Po delta region in northern sharon, Hb Hasharon is probably linked to Italy) who was heterozygous for Hb Haa normal a gene. In comparing Hb Hasharon and a-thalassemia, did not synthesharon’s behavior with that of other a size any normal HbA, and had 3% HbH on variants, particularly HbG Philadelphi...

Certain aspects of the metabolism of centrifuged young and old erythrocytes in hemoglobin H disease have been examined and compared with similar studies of beta thalassemia and normal cells. Glycolysis, hexose monophosphate shunt activity (HMPS), potassium flux, and glutathione (GSH) content were measured. The distributions of hemoglobins H and F, as well as the activities of erythrocyte glucos...

potent induction of fetal hemoglobin (hbf) production results in alleviating the complications of β-thalassemia and sickle cell disease (scd). hbf inducer agents can trigger several molecular signaling pathways critical for erythropoiesis. janus kinase/signal transducer and activator of transcription (jak/stat), mitogen activated protein kinas (mapk) and phosphoinositide 3-kinase (pi3k) are con...

hemoglobin (hb) h disease is a moderate form of α- thalassemia resulting from various genetic defects. hbh disease is not necessarily a benign disorder as has been generally thought. we present hereby a 25- year-old iranian pregnant woman whom referred to our hospital for blood transfusion. she exhibited the clinical and hematological manifestation of hbh disease. her father carries a common α-...

Hemoglobin H (Hb H) disease is the most common form of thalassemia intermedia and has many features that require careful consideration in management. In the majority of cases, Hb H disease results from double heterozygosity for alpha(0)-thalassemia due to deletions that remove both linked alpha-globin genes on chromosome 16, and deletional alpha(+)-thalassemia from single alpha-globin gene dele...