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.

单词	alpha-chain disease
释义	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.
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