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.

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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).

See: Feature record | Search on this feature

Nonspherocytic hemolytic anemia

MedGen UID:: 871250
•Concept ID:: C4025735
•: Disease or Syndrome

See: Feature record | Search on this feature

Abnormality of blood and blood-forming tissues

Term Hierarchy

GTR
MeSH
Orphanet

CClinical test, RResearch test, OOMIM, GGeneReviews, VClinVar

CROGVHeinz body anemia

Phenotypic abnormality
- Abnormality of blood and blood-forming tissues
  - Abnormal erythrocyte morphology
    - Anemia
      - Hemolytic anemia
        Heinz 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).

See: Condition Record

Heinz body anemia

Professional guidelines

PubMed

Treatment of the thalassemia syndrome with splenectomy.

Yang XY, Qu Q, Yang TY, Chan WC, Chu JX, Chen Z, Ning AL, Liu FJ, Lin ZX, Zhou YL
Hemoglobin 1988;12(5-6):601-8. doi: 10.3109/03630268808991650. PMID: 3209402

Hemolysis in the newborn infant resulting from deficiencies of red blood cell enzymes: diagnosis and management.

Gilman PA
J Pediatr 1974 May;84(5):625-34. doi: 10.1016/s0022-3476(74)80001-6. PMID: 4595094

Enzyme-deficient hemolytic anemias. Mechanisms, diagnosis and treatment.

Keitt AS
Mod Treat 1971 May;8(2):402-18. PMID: 4940520

See all (6)

Recent clinical studies

Etiology

Early postnatal diagnosis of hereditary spherocytosis by combining light microscopy, acidified glycerol lysis test and eosin-5'-maleimide binding assay.

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

See all (1)

Diagnosis

Early postnatal diagnosis of hereditary spherocytosis by combining light microscopy, acidified glycerol lysis test and eosin-5'-maleimide binding assay.

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

Salicylazosulphapyridine-induced Heinz body anemia.

Kaplinsky N, Frankl O
Acta Haematol 1978;59(5):310-4. doi: 10.1159/000207777. PMID: 25555

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Therapy

Acute Heinz-body anemia due to severe cresol poisoning: successful treatment with erythrocytapheresis.

Côté MA, Lyonnais J, Leblond PF
Can Med Assoc J 1984 May 15;130(10):1319-22. PMID: 6722696 Free PMC Article

Salicylazosulphapyridine-induced Heinz body anemia.

Kaplinsky N, Frankl O
Acta Haematol 1978;59(5):310-4. doi: 10.1159/000207777. PMID: 25555

See all (2)

Clinical prediction guides

Heinz-body anemia: "bite cell" variant--a light and electron microscopic study.

Ward PC, Schwartz BS, White JG
Am J Hematol 1983 Sep;15(2):135-46. doi: 10.1002/ajh.2830150205. PMID: 6613984

Unstable hemoglobins, hemoglobins with altered oxygen affinity, and m-hemoglobins.

Vichinsky EP, Lubin BH
Pediatr Clin North Am 1980 May;27(2):421-8. doi: 10.1016/s0031-3955(16)33859-7. PMID: 7383714

An ultrastructural study of the red pulp of the spleen and the liver in unstable hemoglobin hemolytic anemia.

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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Synonyms:	Heinz body anemias; Heinz body hemolytic anemia

Genes (locations): Gene(s) directly associated with this condition or phenotype.	HBA1 (16p13.3); HBA2 (16p13.3); HBB (11p15.4)

HPO:	HP:0005511
Monarch Initiative:	MONDO:0007705
OMIM®:	140700

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Heinz body anemia

Definition

Clinical features

Term Hierarchy

Conditions with this feature

Professional guidelines

PubMed

Recent clinical studies

Etiology

Diagnosis

Therapy

Clinical prediction guides

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