Pulmonary mucosa-associated lymphoid tissue lymphoma in a patient with common variable immunodeficiency syndrome

Respiration. 2001;68(1):109-12. doi: 10.1159/000050475.

Abstract

Common variable immunodeficiency syndrome (CVID) is a primary immunodeficiency typically presenting with recurrent sinopulmonary infections. Non-Hodgkin's lymphoma and other secondary cancers are typical late complications of CVID. We report on a patient suffering from CVID with a history of recurrent sinopulmonary infections, interstitial pulmonary changes and hepatic granulomas. Despite treatment with intravenous immunoglobulin followed by a reduction in the number of pulmonary infections, reticular and nodular lung changes progressed. Video-assisted thoracoscopic lung biopsy showed a low-grade B cell lymphoma of the mucosa-associated lymphoid tissue (MALT) of the bronchus without evidence of pulmonary infection. In conclusion, MALT lymphoma of the lung should be considered in the differential diagnosis of progressive lung disease in CVID.

Publication types

  • Case Reports

MeSH terms

  • Adult
  • Biopsy, Needle
  • Chlorambucil / administration & dosage
  • Common Variable Immunodeficiency / complications*
  • Common Variable Immunodeficiency / diagnosis
  • Common Variable Immunodeficiency / drug therapy*
  • Diagnosis, Differential
  • Drug Therapy, Combination
  • Female
  • Follow-Up Studies
  • Humans
  • Immunoglobulins, Intravenous / administration & dosage
  • Lung / pathology*
  • Lymphoma, B-Cell, Marginal Zone / complications*
  • Lymphoma, B-Cell, Marginal Zone / diagnosis*
  • Lymphoma, B-Cell, Marginal Zone / drug therapy
  • Mucous Membrane / pathology
  • Prednisone / administration & dosage
  • Respiratory Function Tests
  • Syndrome
  • Thoracoscopy / methods
  • Tomography, X-Ray Computed

Substances

  • Immunoglobulins, Intravenous
  • Chlorambucil
  • Prednisone