Primary immunodeficiency with hypogammaglobulinemia and minimum midline defect

Abstract

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

Case Report: 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.

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

Keywords: Primary immunodeficiency; Antibody deficiency; Hypogammaglobulinemia; Asthma; Midline defect.