alpha heavy chain disease

alpha heavy chain disease

Seligmann's disease The most common heavy chain disease–paraproteinemia, in which there is an excess production of an incomplete IgA₁–partial heavy chain, no light chain; AHCD affects Sephardic Jews, Arabs, Mediterranean rim, South America, Asia Clinical Onset in childhood or adolescence as a lymphoproliferative disorder in the respiratory or GI tracts with severe diarrhea, malabsorption, steatorrhea, weight loss, hepatic dysfunction, lymphadenopathy, and marked mononuclear infiltration, which may evolve to lymphoma; AHCD may remit spontaneously, respond to antibiotics or, if monoclonal, require combination chemotherapy Lab ↑ Alk phos, ↓ Ca2+ Management Antibiotics, chemotherapy Prognosis May cause death by age 20-30. See IPSID, Mediterranean lymphoma.