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Heinz body anemia
Pathology:
- non-spherocytic hemolytic anemia of Dacie type 1
- 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 result from hemoglobinopathy
- the hemoglobin demonstrates heat lability
Genetics:
- associated with defects in defects in HBA1/HBA2 (hemoglobin-alpha)
Laboratory:
- alpha thalassemia genotyping
- HBA1 gene mutation
- HBA2 gene mutation
- HBA2 gene deletion
Related
Heinz body
General
hemolytic anemia
Database Correlations
OMIM 140700
References
- OMIM :accession 140700
- UniProt :accession P6990