Orphanet: Alpha heavy chain disease

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Alpha-heavy chain disease

Disease 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.


Classification level: Subtype of disorder
  • Synonym(s):
    • Alpha-HCD
    • IPSID
    • Immunoproliferative small intestinal disease
    • Mediterranean lymphoma
  • Prevalence: Unknown
  • Inheritance: -
  • Age of onset: Adolescent, Adult
  • ICD-10: C88.3
  • OMIM: -
  • UMLS: C0021071
  • MeSH: -
  • GARD: -
  • MedDRA: -

Detailed information


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