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Ataxia-pancytopenia syndrome

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Disease definition

A rare genetic disease characterized by cerebellar ataxia, cytopenias and predisposition to bone marrow failure and myeloid leukaemia. Neurologic features variably include slowly progressive cerebellar ataxia or balance impairment with cerebellar atrophy and periventricular white matter T2 hyperintensities in brain MRI, horizontal and vertical nystagmus, dysmetria, dysarthria, pyramidal tract signs and reduced nerve conduction velocity. Hematological abnormalities are variable and may be intermittent and include cytopenias of all cell lineages, immunodeficiency, myelodysplasia and acute myeloid leukemia.

ORPHA:2585

Classification level: Disorder

Synonym(s):
  • ATXPC syndrome
  • SAMD9L-related ataxia-pancytopenia syndrome
  • Myelocerebellar disorder

Prevalence: <1 / 1 000 000

Inheritance: Autosomal dominant

Age of onset: Adolescent, Adult, Childhood

ICD-10: D61.0

OMIM: 159550

UMLS: C1327919

MeSH: C563233

GARD: 3865

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

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

Guidelines
Emergency guidelines
Français (2022.pdf) - Orphanet Urgences
Disease review articles
Review article
English (2025) - Blood
Clinical genetics review
English (2021) - GeneReviews
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.