Agammaglobulinemia acquired D80.1, D83.0, D83.2 D83.8 D83.9

Author: Prof. Dr. med. Peter Altmeyer

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Last updated on: 29.10.2020

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Synonym(s)

acquired agammaglobulinemia; Immune deficiency syndrome Acquired immune deficiency syndrome; Immunodeficiency

Definition
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Family history of antibody deficiency syndrome in young girls and older women with a reduction in IgA, IgG and IgM.

Clinical features
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Diagnosis
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IgA, IgG and IgM in the serum is reduced.

Therapy
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Gamma-globulin substitution (e.g. Intratect) 0.1-0.8 g/kg bw/day every 3-4 weeks, initially start with double the dose (IgG level in plasma should be at least 2 g/l), if necessary prophylactically antibiotics.

Note(s)
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The clinical picture described here is probably identical with the variable immunodeficiency syndrome.

Literature
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  1. Al-Herz W et al (2003) Antibody response in common variable immunodeficiency. Ann Allergy Asthma Immunol 90: 244-247
  2. Barton JC et al (2003) Common variable immunodeficiency and IgG subclass deficiency in central Alabama hemochromatosis probands homozygous for HFE C282Y. Blood Cells Mol Dis 31: 102-111
  3. Sewell WA et al (2003) Therapeutic strategies in common variable immunodeficiency. Drugs 63: 1359-1371
  4. Sidwell RU et al (2002) A case of common variable immunodeficiency presenting with furunculosis. Br J Dermatol 147: 364-367

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Last updated on: 29.10.2020