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Sickle cell S-E disease

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Disease definition

A rare, genetic hemoglobinopathy usually characterized by mild microcytic hemolysis and, very rarely, vaso-occlusive complications. Severe manifestations have been reported, including hematuria, splenic infarction, acute chest syndrome, acute episodes of pain and reversible bone marrow necrosis. The genotype is characterized by an HbS allele in combination with an HbE variant (beta26glu>lys); symptoms are due to the low allelic expression of HbE leading to HbS predominance (65+/-5%).

ORPHA:251375

Classification level: Subtype of disorder

Synonym(s):
  • HbSE disease
  • Hemoglobin S-E disease
  • Sickle cell-hemoglobin E disease

Source: PubMed ID 34134694

Prevalence: Unknown

Inheritance: Autosomal recessive

Age of onset: All ages

ICD-10: D57.2

ICD-11: 3A51.3

UMLS: C1112747

A summary on this disease is available in Français, Español, Deutsch, Italiano, Nederlands
Detailed information

Logo ERN: produced/endorsed by ERN(s) Logo FSMR: produced/endorsed by FSMR(s)

General public
Article for general public
Deutsch (2010) - Onkopedia
Guidelines
Emergency guidelines
Français (2021.pdf) - Orphanet Urgences
Anesthesia guidelines
Deutsch (2014) - Orphananesthesia
English (2014) - Orphananesthesia
Español (2014) - Orphananesthesia
Čeština (2014) - Orphananesthesia
Clinical practice guidelines
English (2015) - Eur J Hum Genet Logo ERN
English (2011) - Am J Hematol Logo ERN
English (2010) - Br J Haematol Logo ERN
Disease review articles
Clinical genetics review
English (2025) - GeneReviews
Disability
Disability factsheet
Genetic testing
Guidance for genetic testing
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