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Sickle cell-beta-thalassemia disease

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Disease definition

A rare, genetic hemoglobinopathy that affects red blood cells both in the production of abnormal hemoglobin, as well as the decreased synthesis of beta globin chains. Clinical manifestations depend on the amount of residual beta globin chains production, and are similar to sickle cell disease, including anemia, vascular occlusion and its complications, acute episodes of pain, acute chest syndrome, pulmonary hypertension, sepsis, ischemic brain injury, splenic sequestration crisis and splenomegaly.

ORPHA:251359

Classification level: Disorder

Synonym(s):
  • HbS-beta-thalassemia syndrome

Source: PubMed ID 21250876

Prevalence: Unknown

Inheritance: Autosomal recessive

Age of onset: All ages

ICD-10: D57.2

ICD-11: 3A51.3 3A51.4

UMLS: C0221019

GARD: 10333

MedDRA: 10040655

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
The documents contained in this website are presented for information purposes only. The material is in no way intended to replace professional medical care by a qualified specialist and should not be used as a basis for diagnosis or treatment.