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Congenital sideroblastic anemia-B-cell immunodeficiency-periodic fever-developmental delay syndrome

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Disease definition

A form of constitutional sideroblastic anemia characterized by severe microcytic anemia, B-cell lymphopenia , panhypogammaglobulinemia and variable neurodegeneration. The disease presents in infancy with recurrent febrile illnesses, gastrointestinal disturbances, developmental delay, seizures, ataxia and sensorineural deafness.

ORPHA:369861

Classification level: Disorder

Synonym(s):
  • SIFD syndrome

Source: PubMed ID 23553769 25193871 36937953

Prevalence: <1 / 1 000 000

Inheritance: Autosomal recessive

Age of onset: Infancy, Neonatal

ICD-10: D64.0

ICD-11: 3A72.01

OMIM: 616084

UMLS: C4707403

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

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

Guidelines
Emergency guidelines
Français (2020.pdf) - Orphanet Urgences
Clinical practice guidelines
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