Sickle cell-hemoglobin D disease syndrome

Orpha code: 251370OMIM code:

Definition

A rare, genetic hemoglobinopathy characterized by all the characteristics of sickle cell anemia (SCA). Clinical course is similar to SCA, including acute episodes of pain, splenic infarction and splenic sequestration crisis, vaso-occlusive crisis, acute chest syndrome, ischemic brain injury, osteomyelitis and avascular bone necrosis. The genotype is characterized by an HbS allele in combination with the HbD variant, beta121Glu>Gln.

Disease data
Classification

Disease

Synonyms
HbSD disease
Choroba HbSD
ORPHA code
251370
OMIM code
-
ICD10 code
D57.2
ICD11 code
3A51.3

No additional description.

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