alpha heavy-chain disease

(redirected from Seligmann’s 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.

Increased alk phos, decreased Ca2+.

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