alpha-chain disease

α-chain dis·ease

a vague or indefinite term; could be used for α-heavy-chain disease (a lymphoplasma cell proliferative disease usually seen in men of Mediterranean descent, characterized by intestinal involvement with steatorrhea, often progressive with fatal outcome) or α-thalassemia (a genetic abnormality in the α-globin chain of hemoglobin).

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.