Lymphoma of mucosa-associated lymphoid tissue in common variable immunodeficiency

Common variable immunodeficiency (CVID) is the most common symptomatic primary immunodeficiency characterized by reduced levels of all major immunoglobuline classes and recurrent c infections. The risk of non-Hodgkin's lymphoma (NHL) among patients with CVID was found to be increased in differe...

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Bibliographic Details
Published in:Leukemia & lymphoma 2006-02, Vol.47 (2), p.343-346
Main Authors: Aghamohammadi, Asghar, Parvaneh, Nima, Tirgari, Farrokh, Mahjoob, Fatemeh, Movahedi, Masoud, Gharagozlou, Mohammad, Mansouri, Mahboubeh, Kouhi, Ali, Rezaei, Nima, Webster, David
Format: Article
Language:English
Subjects:
Adolescent
Biopsy
Common Variable Immunodeficiency - complications
Common Variable Immunodeficiency - diagnosis
Common Variable Immunodeficiency - drug therapy
Diagnosis, Differential
Female
Humans
Immunoglobulins, Intravenous - administration & dosage
Immunoglobulins, Intravenous - therapeutic use
Lung - pathology
Lymphoma, B-Cell, Marginal Zone - complications
Lymphoma, B-Cell, Marginal Zone - diagnosis
Lymphoma, B-Cell, Marginal Zone - drug therapy
Prednisolone - therapeutic use
Remission Induction
Treatment Outcome
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Summary:Common variable immunodeficiency (CVID) is the most common symptomatic primary immunodeficiency characterized by reduced levels of all major immunoglobuline classes and recurrent c infections. The risk of non-Hodgkin's lymphoma (NHL) among patients with CVID was found to be increased in different studies. Mucosa-associated lymphoid tissue (MALT) lymphomas are a recently recognized sub-set of low-grade B-cell NHL composed of marginal zone-related cells. MALT lymphomas appear in the lymphoid tissues as a result of chronic inflammatory or autoimmune stimulation. This study briefly reviews previously published cases and reports a patient suffering from CVID with a history of chronic diarrhea and recurrent sinopulmonary infections. Despite treatment with intravenous immunoglobulin, chronic cough and wheezing progressed. Open lung biopsy showed a MALT lymphoma. Although a rare complication, pulmonary low grade B-cell lymphoma is a diagnosis that must be kept in mind in CVID patients with chronic pulmonary symptoms unresponsive to conventional therapies.
ISSN:1042-8194
1029-2403
DOI:10.1080/10428190500285285