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Hemoglobin Barts

MedGen UID:
29772
•Concept ID:
C0062251
•
Amino Acid, Peptide, or Protein; Biologically Active Substance
Synonyms: Hb Bart's; hemoglobin Bart's; Hemoglobin Bart's; hemoglobin Barts; methemoglobin Bart's
SNOMED CT: Hemoglobin Bart (5830004); Hemoglobin Barts (5830004); Hb - Hemoglobin Barts (5830004)
 
HPO: HP:0005507

Definition

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. [from HPO]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVHemoglobin Barts

Professional guidelines

PubMed

Neonatal screening for α-thalassemia by cord hemoglobin Barts: how effective is it?
Wu MY, Xie XM, Li J, Li DZ
Int J Lab Hematol 2015 Oct;37(5):649-53. Epub 2015 May 8 doi: 10.1111/ijlh.12376. PMID: 25955662
A fast hemoglobin variant on newborn screening is associated with alpha-thalassemia trait.
Miller ST, Desai N, Pass KA, Rao SP
Clin Pediatr (Phila) 1997 Feb;36(2):75-8. doi: 10.1177/000992289703600203. PMID: 9118593

Recent clinical studies

Etiology

Epidemiology of clinically significant forms of alpha- and beta-thalassemia: A global map of evidence and gaps.
Musallam KM, Lombard L, Kistler KD, Arregui M, Gilroy KS, Chamberlain C, Zagadailov E, Ruiz K, Taher AT
Am J Hematol 2023 Sep;98(9):1436-1451. Epub 2023 Jun 26 doi: 10.1002/ajh.27006. PMID: 37357829
Early Evaluation of the Fetal Heart.
Hernandez-Andrade E, Patwardhan M, Cruz-Lemini M, Luewan S
Fetal Diagn Ther 2017;42(3):161-173. Epub 2017 Jul 5 doi: 10.1159/000477564. PMID: 28675906
Clinical manifestations of α-thalassemia.
Vichinsky EP
Cold Spring Harb Perspect Med 2013 May 1;3(5):a011742. doi: 10.1101/cshperspect.a011742. PMID: 23543077Free PMC Article
Alpha and beta thalassemia.
Muncie HL Jr, Campbell J
Am Fam Physician 2009 Aug 15;80(4):339-44. PMID: 19678601
Thalassemia.
Cohen AR, Galanello R, Pennell DJ, Cunningham MJ, Vichinsky E
Hematology Am Soc Hematol Educ Program 2004:14-34. doi: 10.1182/asheducation-2004.1.14. PMID: 15561674

Diagnosis

Epidemiology of clinically significant forms of alpha- and beta-thalassemia: A global map of evidence and gaps.
Musallam KM, Lombard L, Kistler KD, Arregui M, Gilroy KS, Chamberlain C, Zagadailov E, Ruiz K, Taher AT
Am J Hematol 2023 Sep;98(9):1436-1451. Epub 2023 Jun 26 doi: 10.1002/ajh.27006. PMID: 37357829
Early Evaluation of the Fetal Heart.
Hernandez-Andrade E, Patwardhan M, Cruz-Lemini M, Luewan S
Fetal Diagn Ther 2017;42(3):161-173. Epub 2017 Jul 5 doi: 10.1159/000477564. PMID: 28675906
Update in the genetics of thalassemia: What clinicians need to know.
Shang X, Xu X
Best Pract Res Clin Obstet Gynaecol 2017 Feb;39:3-15. Epub 2016 Oct 26 doi: 10.1016/j.bpobgyn.2016.10.012. PMID: 27876354
Clinical manifestations of α-thalassemia.
Vichinsky EP
Cold Spring Harb Perspect Med 2013 May 1;3(5):a011742. doi: 10.1101/cshperspect.a011742. PMID: 23543077Free PMC Article
Alpha and beta thalassemia.
Muncie HL Jr, Campbell J
Am Fam Physician 2009 Aug 15;80(4):339-44. PMID: 19678601

Therapy

Second trimester maternal serum alpha-fetoprotein (MSAFP) as predictor of fetal hemoglobin Bart's disease.
Tongprasert F, Srisupundit K, Luewan S, Tongsong T
Prenat Diagn 2014 Dec;34(13):1277-82. Epub 2014 Aug 22 doi: 10.1002/pd.4465. PMID: 25046597
Second trimester maternal serum markers and a predictive model for predicting fetal hemoglobin Bart's disease.
Tongprasert F, Srisupundit K, Luewan S, Tongsong T
J Matern Fetal Neonatal Med 2013 Jan;26(2):146-9. Epub 2012 Oct 10 doi: 10.3109/14767058.2012.730583. PMID: 22989193
Thalassemia.
Cohen AR, Galanello R, Pennell DJ, Cunningham MJ, Vichinsky E
Hematology Am Soc Hematol Educ Program 2004:14-34. doi: 10.1182/asheducation-2004.1.14. PMID: 15561674
Placental thickness in the first half of pregnancy.
Tongsong T, Boonyanurak P
J Clin Ultrasound 2004 Jun;32(5):231-4. doi: 10.1002/jcu.20023. PMID: 15124189
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 Jul;28(1):105-7. doi: 10.1038/sj.bmt.1703104. PMID: 11498754

Prognosis

Epidemiology of clinically significant forms of alpha- and beta-thalassemia: A global map of evidence and gaps.
Musallam KM, Lombard L, Kistler KD, Arregui M, Gilroy KS, Chamberlain C, Zagadailov E, Ruiz K, Taher AT
Am J Hematol 2023 Sep;98(9):1436-1451. Epub 2023 Jun 26 doi: 10.1002/ajh.27006. PMID: 37357829
Placenta-Derived Extracellular Vesicles in Pregnancy Complications and Prospects on a Liquid Biopsy for Hemoglobin Bart's Disease.
Chaemsaithong P, Luewan S, Taweevisit M, Chiangjong W, Pongchaikul P, Thorner PS, Tongsong T, Chutipongtanate S
Int J Mol Sci 2023 Mar 16;24(6) doi: 10.3390/ijms24065658. PMID: 36982732Free PMC Article
Clinical manifestations of α-thalassemia.
Vichinsky EP
Cold Spring Harb Perspect Med 2013 May 1;3(5):a011742. doi: 10.1101/cshperspect.a011742. PMID: 23543077Free PMC Article
Weighing the evidence for newborn screening for Hemoglobin H disease.
Kemper AR, Knapp AA, Metterville DR, Comeau AM, Green NS, Perrin JM
J Pediatr 2011 May;158(5):780-3. Epub 2010 Dec 16 doi: 10.1016/j.jpeds.2010.10.042. PMID: 21167500
Thalassemia.
Cohen AR, Galanello R, Pennell DJ, Cunningham MJ, Vichinsky E
Hematology Am Soc Hematol Educ Program 2004:14-34. doi: 10.1182/asheducation-2004.1.14. PMID: 15561674

Clinical prediction guides

Epidemiology of clinically significant forms of alpha- and beta-thalassemia: A global map of evidence and gaps.
Musallam KM, Lombard L, Kistler KD, Arregui M, Gilroy KS, Chamberlain C, Zagadailov E, Ruiz K, Taher AT
Am J Hematol 2023 Sep;98(9):1436-1451. Epub 2023 Jun 26 doi: 10.1002/ajh.27006. PMID: 37357829
Placenta-Derived Extracellular Vesicles in Pregnancy Complications and Prospects on a Liquid Biopsy for Hemoglobin Bart's Disease.
Chaemsaithong P, Luewan S, Taweevisit M, Chiangjong W, Pongchaikul P, Thorner PS, Tongsong T, Chutipongtanate S
Int J Mol Sci 2023 Mar 16;24(6) doi: 10.3390/ijms24065658. PMID: 36982732Free PMC Article
Diagnostic value of fetal hemoglobin Bart's for evaluation of fetal α-thalassemia syndromes: application to prenatal characterization of fetal anemia caused by undiagnosed α-hemoglobinopathy.
Singha K, Yamsri S, Chaibunruang A, Srivorakun H, Sanchaisuriya K, Fucharoen G, Fucharoen S
Orphanet J Rare Dis 2022 Feb 10;17(1):45. doi: 10.1186/s13023-022-02197-w. PMID: 35144630Free PMC Article
Placental transcriptome sequencing combined with bioinformatics predicts potential genes and circular RNAs associated with hemoglobin Bart's hydrops fetalis syndrome.
Deng L, Lu Y, Yang D, Yang F, Ruan H, Wei C, Lai K, Pang L
J Obstet Gynaecol Res 2022 Feb;48(2):313-327. Epub 2021 Dec 21 doi: 10.1111/jog.15126. PMID: 34935248
Fetal hemoglobin Bart's hydrops fetalis: pathophysiology, prenatal diagnosis and possibility of intrauterine treatment.
Jatavan P, Chattipakorn N, Tongsong T
J Matern Fetal Neonatal Med 2018 Apr;31(7):946-957. Epub 2017 Mar 21 doi: 10.1080/14767058.2017.1301423. PMID: 28277912

Recent systematic reviews

Epidemiology of clinically significant forms of alpha- and beta-thalassemia: A global map of evidence and gaps.
Musallam KM, Lombard L, Kistler KD, Arregui M, Gilroy KS, Chamberlain C, Zagadailov E, Ruiz K, Taher AT
Am J Hematol 2023 Sep;98(9):1436-1451. Epub 2023 Jun 26 doi: 10.1002/ajh.27006. PMID: 37357829
Weighing the evidence for newborn screening for Hemoglobin H disease.
Kemper AR, Knapp AA, Metterville DR, Comeau AM, Green NS, Perrin JM
J Pediatr 2011 May;158(5):780-3. Epub 2010 Dec 16 doi: 10.1016/j.jpeds.2010.10.042. PMID: 21167500

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