/alpha-heavy chain disease

alpha-heavy chain disease

Rare Disease

MONDO:0015045

Also Known As

Alpha heavy chain diseaseAlpha-HCDIPSIDImmunoproliferative small intestinal diseaseMediterranean abdominal lymphomaMediterranean lymphomaMediterraneanl lymphomaalpha chain diseaseSeligmann's disease

Definition

Alpha-heavy chain disease (alpha-HCD) is 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.