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-...

BACKGROUND Normally, one pair of each of the two alpha-globin genes, alpha1 and alpha2, resides on each copy of chromosome 16. In hemoglobin H disease, three of these four alpha-globin genes are affected by a deletion, a mutation, or both. We studied the alpha1-globin gene abnormalities and the clinical and hematologic features of Chinese patients with hemoglobin H disease in Hong Kong. METHO...

A 19-year old Thai male presented to the hospital with fever, acute hemolysis, pallor and jaundice without hepatosplenomegaly. On admission his hematocrit was 17.4% and a blood smear showed moderate hypochromia with mild anisopoikilocytosis. Hemoglobin (Hb) electrophoresis revealed Hb A2ABart's Hb H with an abnormal band, which on PCR proved to be Hb Pyrgos (beta83, glycine --> aspartic acid). ...

OBJECTIVE Alpha-thalassemia is frequently encountered in eastern Saudi Arabia. We wanted to find out laboratory based incidence and laboratory features of Hemoglobin H disease in the Dammam region. METHODS We retrospectively analyzed the results of Hemoglobin electrophoresis carried out during the last 5 years in our laboratory. Hemoglobin electrophoresis was performed on cellulose acetate, p...

BACKGROUND Early diagnosis during newborn screening or infancy has enabled the observation of the natural history of hemoglobin H disease, a subtype of α-thalassemia. METHODS We analyzed longitudinal clinical data for patients with hemoglobin H disease arising from the deletion of three of four α-globin genes (HbH) and from hemoglobin H Constant Spring (HCS), caused by the deletion of two α-g...

OBJECTIVES Published data indicate that Alpha thalassemia trait is prevalent in 45% of population of Sultanate of Oman. Recent unpublished data suggest that this prevalence is higher than 45%. Yet clinical suspicion or investigations into α-thalassemias are lacking. Moreover, Hemoglobin H disease is considered rare in Oman. We decided, therefore to look for Hemoglobin H disease and characterize...

The recent report on “The Adverse Effects of Pregnancies Complicated by Hemoglobin H (HBH) Disease” is very interesting (1). Rabiee et al. reported a pregnant case complicated with HBH disease. Indeed, this problem might not common in the Middle East but it is very common in Southeast Asia. The authors hereby would like to share the experience on this topic. In the recent report by Tongsong et ...