Alpha-heavy chain disease

Orpha code: 100025OMIM code:

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.

Disease data
Classification

Clinical subtype

Synonyms
Alpha-HCD
Alfa-HCD
Chłoniak śródziemnomorski
Immunoproliferacyjna choroba jelita cienkiego
IPSID
IPSID
Immunoproliferative small intestinal disease
Mediterranean lymphoma
ORPHA code
100025
OMIM code
-
ICD10 code
C88.3
ICD11 code
2A84.0

No additional description.

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