28 Questions
What is the primary consequence of an immunodeficiency?
Infection
What is the estimated prevalence of IgA deficiency in the U.S.?
1 in 300 - 500 people
Which of the following is a characteristic of primary immunodeficiencies?
Always genetic or inherited
What is the primary distinction between primary and secondary immunodeficiencies?
Primary immunodeficiencies are the primary problem, whereas secondary immunodeficiencies are caused by an underlying condition
At what age can primary immunodeficiencies present?
At any age
What is the estimated prevalence of non-IgA deficiency primary immunodeficiencies in live births?
1 in 1200 live births
What is the hallmark of Primary Immunodeficiency Disease (PID)?
Increased susceptibility to infection
What is the characteristic of the blood vessels in Ataxia-Telangiectasia?
Small and dilated
What is the typical presentation of infections in patients with Primary Immunodeficiency Disease (PID)?
Chronic or recurrent 'routine' infections
What is a common feature of DiGeorge Syndrome?
Abnormal facies
What is a possible indication of a mechanical issue in a patient with recurrent infections?
Recurrent infections in the same location
What is a characteristic of Wiskott-Aldrich Syndrome?
Variable B- and T- cell dysfunction
What is the characteristic of Severe Combined Immunodeficiency (SCID)?
Virtually no T cells or immune function
What is the treatment for Severe Combined Immunodeficiency (SCID)?
Bone marrow transplant
What type of disorders are characterized by the presence or absence of T-cells?
T-cell and Combined Defects
What is the name of the syndrome that is caused by a defect in the C3 protein?
S.pneumonia H
What is the age of presentation for Severe Combined Immunodeficiency (SCID)?
Within the first year of life
What is the most common phagocytic disorder?
Chronic granulomatous disease
Which laboratory test is used to diagnose complement deficiency?
CH50 Assay
What is the most common type of cancer in patients with PID?
Non-Hodgkin lymphoma
What is a common symptom of chronic granulomatous disease?
Painful sores around the mouth
What is the estimated cancer risk for PID patients?
4-25%
Which of the following is used to evaluate suspected T-cell disorders?
Lymphocyte subset analysis
What is the origin of Non-Hodgkin lymphoma in patients with PID?
B-cell
Which of the following PIDs has the highest incidence of cancers, including Hodgkin and Non-Hodgkin lymphoma?
Ataxia-telangiectasia
What is a symptom that patients with PID should be educated to report?
Recurrent fever
What is a general recommendation for cancer prevention in patients with PID?
Avoid STIs
In which tissues are Non-Hodgkin lymphoma in patients with PID often found?
Gastrointestinal tract and central nervous system
Test your knowledge of primary immunodeficiencies, including their differences from secondary immunodeficiencies, clinical presentations, and associations with autoimmune diseases. Identify patients who should be evaluated and describe the broad categories of PIDs.
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