alpha heavy chain disease


Also found in: Dictionary, Thesaurus, Encyclopedia.
Related to alpha heavy chain disease: mu 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.

alpha heavy chain disease

A form of heavy chain disease that is related to Mediterranean lymphoma and celiac sprue. The principal organ involved is the small intestine, although respiratory tissues are occasionally affected. The symptoms and signs may include malabsorption, diarrhea, abdominal pains, and weight loss. In some patients there is peripheral adenopathy and splenomegaly with no signs of intestinal or respiratory tract changes. Diagnosis is made through tests for the abnormal immunoglobulins. Chemotherapy may produce long-term remissions.
Synonym: Seligmann's disease