Primary B-cell immunodeficiencies

the primary B-cell immunodeficiencies based on the clues provided.
Quiz by electronicmayhem
Last updated: July 18, 2014
First submittedJuly 18, 2014
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most common primary immunodeficiency, increased incidence of allergies and autoimmune disease, 20% of patients will also have IgG subclass deficiencies, most patients have no symptoms
immunoglobulin A deficiency
normal numbers of CD19+ B-cells, low levels of IgG and IgA, presents from infancy to 4th decade, many patients have co-morbid autoimmune disease, increased risk of B-cell lymphomas and gastric carcinoma, patients commonly get respiratory tract infections
common variable immunodeficiency
deficiency of 1 or more IgG subclass, usually normal levels of total IgG
IgG subclass deficiency
normal numbers of CD19+ B-cells, low levels of IgG and IgA, presents around 6 months of age as maternal Ig wanes, increased frequency of upper respiratory infections, often diagnosed retrospectively as a CVID that the child outgrows
transient hypogammaglobulinemia of infancy
defective B-cell maturation due to mutation in B-cell/Bruton tyrosine kinase on chromosome X, presents in boys 6-12 months old with recurrent pyogenic infections from encapsulated bacteria, low levels of IgG with absent IgA and IgM, absence of CD19+ B-cells in peripheral blood, lymph nodes lack germinal centers and plasma cells
Bruton agammaglobulinemia
results from a failure of Ig class swicthing due to a mutation in CD40L (CD145) on T-cells, IgM levels can be normal to elevated while there is absent IgA and IgE and absent to low IgG (maternal leftover), normal numbers of CD19+ B-cells, CD40L- T-cells on flow cytometry, X-linked, presents before 1 year of age
Hyperimmunoglobulin M syndrome
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