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alpha-Thalassemia

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A disorder characterized by reduced synthesis of the alpha chains of hemoglobin. The severity of this condition can vary from mild anemia to death, depending on the number of genes deleted.

Also Known As:

A-Thalassemia; A-Thalassemias; Disease, Hemoglobin H; Diseases, Hemoglobin H; Hemoglobin H Diseases; Thalassemia alpha; Thalassemia-alphas; alpha Thalassemia; alpha-Thalassemias; Hemoglobin H Disease; Thalassemia-alpha

Networked: 964 relevant articles (7 outcomes, 102 trials/studies)

Relationship Network

Disease Context: Research Results

Related Diseases

1.	Anemia
2.	Hydrops Fetalis (Fetal Edema)
3.	Sickle Cell Anemia (Hemoglobin S Disease)
4.	Bronchitis
5.	Congenital Abnormalities (Deformity)

Experts

1.	Fucharoen, Supan: 21 articles (01/2015 - 01/2002)
2.	Fucharoen, Goonnapa: 19 articles (01/2015 - 01/2002)
3.	Harteveld, Cornelis L: 18 articles (10/2011 - 08/2002)
4.	Sanchaisuriya, Kanokwan: 17 articles (01/2015 - 01/2002)
5.	Giordano, Piero C: 17 articles (08/2010 - 08/2002)
6.	Fucharoen, Suthat: 14 articles (02/2012 - 05/2002)
7.	Higgs, Douglas R: 10 articles (11/2008 - 02/2002)
8.	Fucharoen, S: 10 articles (10/2003 - 01/2000)
9.	Winichagoon, Pranee: 9 articles (05/2010 - 05/2002)
10.	van Delft, Peter: 9 articles (01/2010 - 08/2002)

Drugs and Biologics

Drugs and Important Biological Agents (IBA) related to alpha-Thalassemia:

1.	DNA (Deoxyribonucleic Acid)IBA 12/01/2009 - "A rare case of thalassaemia-intermedia involving a non-deletion alpha thalassemia point mutation in the alpha1-globin gene CD59 (GGC --> GAC) and a deletion alpha+ (-alpha(3.7)) thalassaemia in which use of high performance liquid chromatography (HPLC) C-gram Hb subtype profile and DNA molecular analysis helped establish the diagnosis." 03/01/2010 - "A blinded study of 110 samples, which included 92 alpha-thalassemia samples with various genotypes and 18 normal DNA samples, was carried out by the methods. " 06/01/2003 - "The gene frequency of alpha thalassemia is likely to be high but requires DNA studies." 05/01/1997 - "Laboratories that perform DNA-based alpha-thalassemia testing in populations similar to the one residing in Washington State should use a testing system that allows for the unequivocal identification of the haplotypes detected in the study population, namely --SEA, -alpha3.7, and --FIL." 10/01/1990 - "A case is reviewed documenting the utilization of DNA studies in managing patients known or suspected to have a history of alpha-thalassemia major."
2.	hemoglobin Bart'sIBA 08/01/2000 - "A reasonably accurate estimate of the alpha-thalassemia carrier frequency may be obtained from cord blood studies if Hb Bart's estimates are combined with hematological indices. " 06/01/2015 - "Newborn screening for hemoglobin H disease is especially appealing because the screening test is based on the detection of hemoglobin Bart's (γ4) that is only possible within the newborn period. " 01/01/2006 - "Preliminary results in quantitative determination of Hb Bart's in Hb solution of 3 alpha thalassemia families having at least 1 child with HbH disease and 6 normal subjects indicated that it was possible to quantify Hb Bart's by our developed ELISA with appropriate sensitivity and specificity." 01/01/2006 - "Hb Bart's in cord blood: an accurate indicator of alpha-thalassemia." 01/01/2004 - "Alpha thalassemia is also being recognized with increasing frequency in North America, and newborn screening for Hemoglobin Barts in some states is leading to early detection of Hb H disease and Hb H Constant Spring. "
3.	IronIBA 09/01/2009 - "Alpha thalassemia trait was presumed if the red blood cell (RBC) count was elevated, no variant Hbs were detected, and the iron studies were within normal ranges. " 08/01/2014 - "Some physicians neglect the possible coexistence of an iron deficiency with a thalassemia minor and do not treat the iron deficiency accordingly. " 01/01/2011 - "However, she was anaemic on admission and subsequent investigations revealed that she had alpha-thalassemia and iron deficiency anaemia. " 01/01/2011 - "An Unusual Association between Unilateral Intracranial Vessels Occlusion with Iron Deficiency Anaemia and Alpha-Thalassemia Trait: A Case Report." 08/01/2010 - "Therefore, to avoid the risk of having affected offspring, carrier detection, genetic counseling, and prenatal diagnosis would be of vital importance for individuals with low red blood cell (RBC) indices, normal iron status, and normal HbA(2) level, who are suspected to be alpha-thalassemia carriers."
4.	Fetal Hemoglobin (Hemoglobin F)IBA 10/29/2009 - "Previous studies have shown that the coinheritance of alpha-thalassemia or the presence of genetic variants that sustain fetal hemoglobin production has a strong impact on ameliorating the clinical phenotype. " 01/01/2005 - "Studies have shown the importance of high hemoglobin F determinants and alpha-thalassemia as modifiers of disease severity, but these alone do not explain the diversity that is seen. " 08/01/2014 - "Fetal hemoglobin and alpha thalassemia modulate the phenotypic expression of HbSD-Punjab." 02/01/2014 - "Genetic characteristics affecting the clinical severity of SCA include variations in the hemoglobin F (HbF) level, coexistence of alpha-thalassemia, and the haplotype associated with the HbS gene. " 02/01/1997 - "There were only two statistically independent associations: alpha thalassemia status and average steady state fetal hemoglobin (HbF). "
5.	Hemoglobin EIBA 11/01/2008 - "All forms of alpha-thalassemia including double heterozygote for hemoglobin E and alpha-thalassemia showed significant reduction in hemoglobin, mean corpuscular volume, and mean corpuscular hemoglobin with increasing trend of red blood cell as compared with a non-alpha-thalassemic group. " 05/01/2009 - "However, sTfR has never been studied in hemoglobin E (HbE) carriers and their interactions with alpha-thalassemia heterozygotes that are common in Southeast Asia. " 03/01/2005 - "Hemoglobin E levels in double heterozygotes of hemoglobin E and SEA-type alpha-thalassemia." 10/01/2003 - "Molecular and hematologic features of hemoglobin E heterozygotes with different forms of alpha-thalassemia in Thailand." 10/01/2003 - "Interaction of hemoglobin E and several forms of alpha-thalassemia in Cambodian families."
6.	OxygenIBA 01/01/2005 - "Homozygous cases, and patients who are compound heterozygotes for a high oxygen affinity Hb and a thalassemia (thal), are discussed. " 07/01/2006 - "Oxygen uptake kinetics during heavy submaximal exercise: Effect of sickle cell trait with or without alpha-thalassemia." 05/01/1998 - "The aim of the present study was to assess blood lactate concentrations ([LA], mmol x L(-1)) and oxygen uptake (VO2, L x min(-1), mL x kg(-1) x min(-1)) during incremental exercise in subjects with sickle cell trait (SCT) only, i.e., sedentary subjects with SCT without anemia and/or associated alpha thalassemia. " 03/01/2013 - "The hemolytic component was lower with hydroxyurea therapy, higher hemoglobin F, and alpha-thalassemia (P≤0.0005); it was higher with higher systemic pulse pressure, lower oxygen saturation, and greater values for tricuspid regurgitation velocity, left ventricular diastolic dimension and left ventricular mass (all P<0.0001). " 01/01/2010 - "Erythrocytosis due to a combination of the high oxygen affinity hemoglobin variant, Hb Olympia [beta20(B2)Val-->Met] with beta- and alpha-thalassemia mutations: first case in the literature."
7.	zeta-GlobinsIBA 02/01/2008 - "A commercial zeta-globin enzyme-linked immunosorbent assay (ELISA) is effective in detecting Southeast Asian (SEA) alpha-thalassemia. " 09/01/1989 - "A sensitive and specific radioimmunoassay (RIA) for human embryonic zeta-globin chains was used to study normal fetal blood and newborn cord blood as well as cord blood from newborns with alpha-thalassemias. " 03/01/2005 - "A mouse strain carrying an embryonic zeta-globin gene disrupted by the insertion of a PGK-Neo cassette displays an alpha-thalassemia-like syndrome. " 01/01/2002 - "We evaluated an enzyme-linked immunosorbent assay (ELISA) for embryonic zeta-globin chains as a routine screening test for (--(SEA)) alpha-thalassemia deletion (SEA deletion). " 01/01/2002 - "Routine screening of (--(SEA)) alpha-thalassemia deletion by an enzyme-linked immunosorbent assay for embryonic zeta-globin chains."
8.	Hydroxyurea (Hydrea)FDA LinkGeneric 01/01/2009 - "Hydroxyurea was effective in reducing the clinical severity in Indian patients who initially had higher HbF levels and the presence of ameliorating factors, such as alpha-thalassemia and the Xmn I polymorphism. " 07/01/2013 - "At study exit, subjects randomized to placebo still exhibited laboratory effects of alpha thalassemia and other modifiers, while those assigned hydroxyurea had treatment effects that exceeded most genetic influences. " 09/01/2009 - "In group I, clinical response to hydroxyurea was better in patients with alpha-thalassemia, XmnI (+/+) and a higher mean fold increase in gamma mRNA expression. " 06/01/1996 - "After obtaining informed consent, we initiated hydroxyurea therapy at a dosage of 10 to 20 mg/kg per day in 15 patients with severe SCD (hemoglobin SS, hemoglobin SS-alpha thalassemia, or hemoglobin S-beta0-thalassemia). " 03/01/2013 - "The hemolytic component was lower with hydroxyurea therapy, higher hemoglobin F, and alpha-thalassemia (P≤0.0005); it was higher with higher systemic pulse pressure, lower oxygen saturation, and greater values for tricuspid regurgitation velocity, left ventricular diastolic dimension and left ventricular mass (all P<0.0001). "
9.	PolymersIBA 01/01/1989 - "Our analyses of equilibrium polymer formation at physiological oxygen saturation values suggest that small decreases in polymer formation at intermediate levels of hemoglobin F may give rise to a small decrease in anemia (as associated with homozygous alpha-thalassemia coexistent with sickle cell anemia), but that greater reductions in polymer formation may be necessary to effect a significant improvement in disease severity. " 01/01/1985 - "In our studies, the polymer fraction in sickle red cells was determined from reported mean values for hemoglobin composition and mean corpuscular hemoglobin concentration (MCHC) in 12 groups of patients with sickle hemoglobinopathies (homozygotes for HbS, with and without coexistent alpha-thalassemia or various forms of the hereditary persistence of fetal hemoglobin [HPFH], beta+-, beta 0-, and delta beta-thalassemia, and heterozygotes for HbS with HbA). " 02/13/2009 - "To achieve the best CE separation, several parameters were evaluated for optimizing the separation conditions, including the capillary coating, the concentrations of polymer sieving matrix, the sizes and concentrations of GNPs, the buffer concentrations, and the pH. The final CE method for separating a 200-base pair (bp) DNA ladder and alpha-thalassemia deletions used a DB-17 capillary, 0.6% poly(ethylene oxide) (PEO) prepared in a mixture of GNP(32nm) solution and glycine buffer (25mM, pH 9.0) (80:20, v/v) as the sieving matrix with 1microM YO-PRO-1 for fluorescence detection; the applied voltage was -10kV (detector at anode side) and the separation temperature was 25 degrees C. "
10.	CalciumIBA 01/01/2014 - "We should perform annual BMD in patients with thalassemia major and intermedia and hemoglobin H disease in age of higher than 8 year old and treat low BMD with administration of bisphosphonate, calcium and vitamin D supplements. " 08/01/2010 - "Improving access to prenatal care and prenatal calcium and iron supplementation, and screening for congenital abnormalities and alpha-thalassemia may help to reduce rates of perinatal death in Vietnam."

Therapies and Procedures

1.	Prenatal Care 08/01/2010 - "Improving access to prenatal care and prenatal calcium and iron supplementation, and screening for congenital abnormalities and alpha-thalassemia may help to reduce rates of perinatal death in Vietnam."
2.	Ligation 08/15/2010 - "In this study, Multiplex Ligation-dependent Probe Amplification was used to screen for deletions in the telomeric region of the short arm of chromosome 16, in an attempt to explain the alpha-thalassemia or the HbH disease present in a group of Portuguese patients. " 01/01/2010 - "In the present study, for detection of alpha-thalassemia we employed Multiplex-PCR (mPCR) and Multiplex Ligation-dependent Probe Amplification (MPLA). " 11/01/2007 - "In this study, we have fine mapped deletions causing alpha-thalassemia within 2 Mb from the telomere of 16p by multiplex ligation-dependent probe amplification (MLPA). " 01/01/2008 - "Detection of alpha-thalassemia in China by using multiplex ligation-dependent probe amplification." 01/01/2008 - "The multiplex ligation-dependent probe amplification (MLPA) method was used to analyze 118 DNA samples from 90 alpha-thalassemia (alpha-thal) patients and 28 normal persons from Southern China, where the main causes of alpha-thal are three large deletions (-alpha3.7, -alpha4.2, and --SEA) and two point mutations in the alpha-globin gene cluster on chromosome 16. "
3.	Splenectomy 01/01/1997 - "Selection criteria should include a mild beta-genotype of coinheritance of alpha-thalassemia, splenectomy and pretreatment reticulocyte response of the patients as well as the patients' compliance." 10/01/1982 - "Hematologic improvement following splenectomy for hemoglobin-H disease." 10/01/2007 - "The research was conducted to study humoral and cellular immunity of 30 Thalassemia intermedia patients and 15 patients with Hemoglobin H disease in different periods after splenectomy. " 01/01/2013 - "Pulmonary hypertension risk in patients with hemoglobin h disease: low incidence and absence of correlation with splenectomy." 01/01/1989 - "Sixty-nine children with beta-thalassemia/E disease, hemoglobin H disease and beta-thalassemia major who were followed for at least 2 years before and after splenectomy were studied retrospectively for the results of the splenectomy. "
4.	Continuous Ambulatory Peritoneal Dialysis (CAPD) 03/01/1992 - "Four of the 40 randomly selected patients (22 on hemodialysis [HD], 18 on continuous ambulatory peritoneal dialysis [CAPD]) were identified to be thalassemic prior to a trial of rHuEPO (alpha-thalassemia trait in three and beta-thalassemia minor in one). "
5.	Renal Dialysis (Hemodialysis) 03/01/1992 - "Four of the 40 randomly selected patients (22 on hemodialysis [HD], 18 on continuous ambulatory peritoneal dialysis [CAPD]) were identified to be thalassemic prior to a trial of rHuEPO (alpha-thalassemia trait in three and beta-thalassemia minor in one). "