Case SH 431


História clínica

13 year-old girl, born at term from non-consanguineous parents, without infections in her infancy. The patient had cervical lymphadenopathy at 2 years (EBV-positive) that regressed. She later had recurrent bouts of lymphadenopathy, enlarged tonsils and adenoids (with recurrent adenoid hypertrophy after adenoidectomy). Bronchoscopy revealed endobronchial nodules. Nodal marginal zone lymphoma was diagnosed on a subsequent cervical lymph node biopsy. The patient has splenomegaly, bronchiectasis, occasional otitis, 4-5 upper respiratory tract infections per year, several episodes of pneumonia but no severe life threatening infections. There is mild conductive hearing loss bilaterally and persistent asthma since age 2. Serologic studies reveal EBV viremia with a virus load of 3000. CMV, HIV, and HTLV are negative. CBC with differential is within normal limits. Quantitative immunoglobulins (IgG, IgA, IgM, IgE) are normal. Testing revealed a poor response to Pneumovax 23.






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