Types of primary immunodeficiency:
(1) B-lymphocyte
(2) T-lymphocyte
(3) neutrophil or monocyte
(4) complement
Most patients were referred for evaluation by hospital clinicians.
The usual trigger was a child with severe, unusual or recurrent infections.
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Warning signs for a primary immunodeficiency
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Suggests
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family history of an immunodeficiency
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B-cell PID, complement deficiciencies, others
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failure to thrive
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T-cell PID
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use of intravenous antibiotics or antifungal agents to treat sepsis
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neutrophilic deficiencies
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These findings identified the majority of patients but can miss 4 to 11% of patients depending on the type of immunodeficiency.
The initial workup might involve:
(1) quantitative serum immunoglobulin
(2) absolute lymphocyte count with subsets
(3) antibody responsse to vaccination such as tetanus
(4) absolute neutrophil count
(5) neutrophil oxidative burst test
(6) complement assay (CH50, alternative pathway, etc)
