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Hereditary isolated aplastic anemia

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Disease definition

Hereditary isolated aplastic anemia is a rare, genetic, constitutional aplastic anemia disorder characterized by severe peripheral blood pancytopenia and bone marrow hypoplasia in multiple individuals of a family, in the absence of any somatic symptoms. Abnormal bleeding, as well as erythrocyte macrocytosis, is reported and patients usually become transfusion-dependent.

ORPHA:397692

Classification level: Disorder

Prevalence: Unknown

Inheritance: Autosomal dominant, Autosomal recessive

Age of onset: Childhood

ICD-10: D61.0

ICD-11: 3A70.0

OMIM: 616553

UMLS: C5190891

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

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

Guidelines
Emergency guidelines
Français (2022.pdf) - Orphanet Urgences
Clinical practice guidelines
English (2016) - Br J Haematol Logo ERN
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.