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Question 2#

A 20-year-old man is found to have weight loss and generalized lymphadenopathy. He has hypogammaglobulinemia with a normal distribution of immunoglobulin isotypes. Histologic examination of lymphoid tissue shows germinal center hyperplasia. A diagnosis of common variable immunodeficiency is made.

Which of the following statements is correct?

A. The patient likely had symptoms in childhood
B. At least one parent is also afflicted with the disease
C. The patient may develop recurrent bronchitis and chronic idiopathic diarrhea
D. The patient should receive the standard vaccine protocol.
E. The patient should receive trimethoprim-sulfamethoxazole as prophylaxis against Pneumocystis infections

Correct Answer is C


Patients with common variable immunodeficiency (CVI) syndrome usually develop recurrent or chronic infections of the respiratory or gastrointestinal tract. The fundamental feature is hypogammaglobulinemia, often with mild T-cell abnormalities. Diarrhea can be idiopathic, or secondary to malabsorption or chronic infection such as giardiasis. There is no mendelian genetic inheritance, although clusters in families do occur. Symptoms generally do not occur until the second or third decade of life, but also may first present in the older patient. Think of CVI in adults with recurrent sinusitis, bronchitis, pneumonia, or GI symptoms even in the absence of childhood history of recurrent infections. Patients with common variable immunodeficiency syndrome should not receive live vaccines such as MMR, varicella, or oral polio vaccines. Despite their subtle T-cell defects, patients with common variable immunodeficiency are rarely infected with organisms that afflict T-cell– deficient patients (such as patients with HIV).