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Alpha-heavy chain disease

ORPHA:100025· ICD-10 C88.3

Definition

A type of HCD characterized by the production of incomplete monoclonal alpha-heavy chains without associated light chains. Alpha-HCD is considered to be a subtype of immunoproliferative small intestinal disease (IPSID). The clinical presentation includes chronic diarrhea with evidence of malabsorption.

Prevalence
Unknown
Age of onset
Adolescent, Adult