Autoimmune lymphoproliferative…

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Autoimmune lymphoproliferative syndrome-recurrent viral infections due to CASP8 deficiency

ORPHA:275517Disease

Also called ALPS-recurrent viral infections due to CASP8 deficiency · Autoimmune lymphoproliferative syndrome-recurrent viral infections due to Caspase 8 deficiency · CEDS · Caspase 8 deficiency syndrome

What it is

A rare genetic disorder characterized by lymphadenopathy and/or splenomegaly and recurrent infections due to herpes viruses.

Key facts

Prevalence
<1 / 1 000 000
Age of onset
Childhood
Inheritance
Autosomal recessive
Classified as
Disease

Prevalence figures describe populations, not individuals, and are often estimates from a single study. Where the figure is an incidence or a birth prevalence, it says so.

Gene

CASP8Disease-causing germline mutation(s)

ICD-10 codes

D47.9filed under a broader ICD-10 category — shared with 4 other rare diseases

ICD-10 has no distinct code for most rare diseases, so many are filed under the nearest available one. A shared code identifies the group, not this disease — the ORPHAcode above is what identifies it.

Related health topics

Reached through the ICD-10 categories this disease is filed under, so these describe the broader group rather than this disease specifically.

Cross-references

GARD 9796MONDO 0011804OMIM 607271UMLS C1846545

Orphadata Science: Free access data from Orphanet. © INSERM 1999. Data version 2026-06-23. Available under CC BY 4.0. This is a reference, not medical advice.

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