[Primary immunodeficiency with hypogammaglobulinemia and minimum midline defect]

In allergic patients with recurrent respiratory infections, the association of antibody deficiencies, both selective and specific to polysaccharide responses, has been described.

Male, no significant family history. At age 5, presented with cervical and axillary lymphadenopathy; biopsy ruled out malignancy, followed by remission. At age 7, developed nasal symptoms and wheezing. Admitted to Allergy at age 9 and diagnosed with asthma and rhinitis, sensitized to dust mites and grass. Good response to immunotherapy in the first 2 years; during the third year, developed recurrent respiratory and gastrointestinal infections every 15 days. At age 13, further workup was decided. Physical Examination: Weight 69 kg, height 1.7 m, obstructive turbinates, central bifid uvula, grade I tonsils, no lymphadenopathy, cardiopulmonary system unremarkable. Studies: Hemoglobin 15.6 g/dL, Hematocrit 46%, WBC 4005/μL, Lymphocytes 1200/μL (20%), Eosinophils 210/μL (3.6%), Platelets 219,000/μL. Low IgA 23 mg/dL (45-236), low IgM 27 mg/dL (52-242), IgE 11.9 IU/mL (≤3100), normal IgG 1060 mg/dL (560-1760), reconfirmed. Flow cytometry normal with normal B lymphocytes. Pneumococcal polysaccharide vaccine challenge showed adequate response to only 4 serotypes (<50% response). Management: Temporary treatment with prophylactic antibiotics and immunostimulants, with improvement in infectious episodes. Currently off prophylaxis. Discussion: Adolescent with quantitative IgA and IgM deficiency, currently normal IgG levels but impaired response to polysaccharide vaccine. Maintained under immunological surveillance due to potential progression to common variable immunodeficiency.

Recurrent or invasive infections in treated asthma patients warrant investigation for antibody deficiency.