Alpha-thalassemia

An inherited hemoglobinopathy characterized by impaired synthesis of alpha-globin chains leading to a variable clinical picture depending on the number of affected alleles.

Hemoglobin Barts

Normal adult hemoglobin is composed of two chains each of alpha and beta globin. Hb Barts (Hemoglobin Barts) is a tetramer with four gamma globin chains, and is essentially pathognomonic for one or another form of alpha thalassemia. Hb Barts has an extremely high affinity for oxygen, resulting in almost no oxygen delivery to the tissues.


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(per page)
PMID (PMCID)
20220267
(2855068)
 MIXED_SAMPLE Infant, Newborn
Homozygosity for the Mediterranean a-thalassemic deletion (hemoglobin Barts hydrops fetalis).
Al-Allawi NA, Shamdeen MY, Rasheed NS.
Ann Saudi Med. 2010;30(2):153-5.
Molecular studies revealed that the fetus had the hemoglobin Barts hydrops fetalis syndrome due to homozygosity for the Mediterranean alpha-thalassemia deletion.
20220267
(2855068)
 MIXED_SAMPLE Infant, Newborn
Homozygosity for the Mediterranean a-thalassemic deletion (hemoglobin Barts hydrops fetalis).
Al-Allawi NA, Shamdeen MY, Rasheed NS.
Ann Saudi Med. 2010;30(2):153-5.
Hemoglobin Barts hydrops fetalis syndrome is the most severe and generally fatal clinical phenotype of alpha-thalassemia.
11498754
 FEMALE Infant
Successful mismatched sibling cord blood transplant in Hb Bart's disease.
Zhou X, Ha SY, Chan GC, Luk CW, Chan V, Hawkins B, Lam YH, Liang RH, Lau YL.
Bone Marrow Transplant. 2001;28(1):105-7.
Ten percent of hemoglobin Bart's was detected 2 months after transplant and this reflects the alpha-thalassemia trait of the donor.
1292309
 MIXED_SAMPLE
Long-term survival of a baby with homozygous alpha-thalassemia-1.
Lam TK, Chan V, Fok TF, Li CK, Feng CS.
Acta Haematol. 1992;88(4):198-200.
The girl with hemoglobin Bart's hydrops syndrome, confirmed by gene analysis to be homozygous for alpha-thalassemia-1, survives for 27 months at the time of reporting.