alpha heavy-chain disease

alpha heavy-chain 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.

Ethnic groups/areas affected
Sephardic Jews, Arabs, Mediterranean rim, South America, Asia.
 
Clinical findings
Onset in childhood or adolescence as a lymphoproliferative disorder in the respiratory or GI tracts with severe diarrhoea, malabsorption, steatorrhoea, 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
Increased alk phos, decreased Ca2+.

Management
Antibiotics, chemotherapy.
 
Prognosis
May cause death by age 20–30.