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Heinz body anemia

MedGen UID:
148583
Concept ID:
C0700299
Disease or Syndrome
Synonyms: Heinz body anemias; Heinz body hemolytic anemia
 
Genes (locations): HBA1 (16p13.3); HBA2 (16p13.3); HBB (11p15.4)
 
HPO: HP:0005511
Monarch Initiative: MONDO:0007705
OMIM®: 140700

Definition

This is a form of nonspherocytic hemolytic anemia of Dacie type I (in vitro autohemolysis is not corrected by added glucose). After splenectomy, which has little benefit, basophilic inclusions called Heinz bodies are demonstrable in the erythrocytes. Before splenectomy, diffuse or punctate basophilia may be evident. Most of these cases are probably instances of hemoglobinopathy. The hemoglobin demonstrates heat lability. Heinz bodies are observed also with the Ivemark syndrome (asplenia with cardiovascular anomalies; 208530). [from OMIM]

Clinical features

From HPO
Heinz bodies
MedGen UID:
42375
Concept ID:
C0018871
Cell Component
A type of erythrocyte inclusion composed of denatured hemoglobin.
Heinz body anemia
MedGen UID:
148583
Concept ID:
C0700299
Disease or Syndrome
This is a form of nonspherocytic hemolytic anemia of Dacie type I (in vitro autohemolysis is not corrected by added glucose). After splenectomy, which has little benefit, basophilic inclusions called Heinz bodies are demonstrable in the erythrocytes. Before splenectomy, diffuse or punctate basophilia may be evident. Most of these cases are probably instances of hemoglobinopathy. The hemoglobin demonstrates heat lability. Heinz bodies are observed also with the Ivemark syndrome (asplenia with cardiovascular anomalies; 208530).
Nonspherocytic hemolytic anemia
MedGen UID:
871250
Concept ID:
C4025735
Disease or Syndrome

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVHeinz body anemia
Follow this link to review classifications for Heinz body anemia in Orphanet.

Conditions with this feature

Heinz body anemia
MedGen UID:
148583
Concept ID:
C0700299
Disease or Syndrome
This is a form of nonspherocytic hemolytic anemia of Dacie type I (in vitro autohemolysis is not corrected by added glucose). After splenectomy, which has little benefit, basophilic inclusions called Heinz bodies are demonstrable in the erythrocytes. Before splenectomy, diffuse or punctate basophilia may be evident. Most of these cases are probably instances of hemoglobinopathy. The hemoglobin demonstrates heat lability. Heinz bodies are observed also with the Ivemark syndrome (asplenia with cardiovascular anomalies; 208530).

Professional guidelines

PubMed

Gilman PA
J Pediatr 1974 May;84(5):625-34. doi: 10.1016/s0022-3476(74)80001-6. PMID: 4595094
Goldstein BD
Am J Med Sci 1974 May;267(5):291-7. doi: 10.1097/00000441-197405000-00005. PMID: 4151976
Keitt AS
Mod Treat 1971 May;8(2):402-18. PMID: 4940520

Recent clinical studies

Etiology

Andres O, Eber S, Speer CP
Ann Hematol 2015 Dec;94(12):1959-64. Epub 2015 Sep 4 doi: 10.1007/s00277-015-2491-z. PMID: 26336967

Diagnosis

Andres O, Eber S, Speer CP
Ann Hematol 2015 Dec;94(12):1959-64. Epub 2015 Sep 4 doi: 10.1007/s00277-015-2491-z. PMID: 26336967
Kaplinsky N, Frankl O
Acta Haematol 1978;59(5):310-4. doi: 10.1159/000207777. PMID: 25555

Therapy

Côté MA, Lyonnais J, Leblond PF
Can Med Assoc J 1984 May 15;130(10):1319-22. PMID: 6722696Free PMC Article
Kaplinsky N, Frankl O
Acta Haematol 1978;59(5):310-4. doi: 10.1159/000207777. PMID: 25555

Clinical prediction guides

Ward PC, Schwartz BS, White JG
Am J Hematol 1983 Sep;15(2):135-46. doi: 10.1002/ajh.2830150205. PMID: 6613984
Vichinsky EP, Lubin BH
Pediatr Clin North Am 1980 May;27(2):421-8. doi: 10.1016/s0031-3955(16)33859-7. PMID: 7383714
Matsumoto N, Ishihara T, Adachi H, Takahashi M, Miwa S
Virchows Arch A Pathol Anat Histol 1977 Jul 11;374(4):339-51. doi: 10.1007/BF00432655. PMID: 142356

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