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 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.
Aghamohammadi
Asghar
A
Department of Clinical Pediatric Immunology, Children's Medical Center Hospital, Tehran University of Medical Sciences, Tehran, Iran. aghamohammadi@iranianpia.org
Parvaneh
Nima
N
Tirgari
Farrokh
F
Mahjoob
Fatemeh
F
Movahedi
Masoud
M
Gharagozlou
Mohammad
M
Mansouri
Mahboubeh
M
Kouhi
Ali
A
Rezaei
Nima
N
Webster
David
D
D000293:Adolescent; D001706:Biopsy; D017074:Common Variable Immunodeficiency / Q000150:complications* / Q000175:diagnosis / Q000188:drug therapy*; D003937:Diagnosis, Differential; D005260:Female; D006801:Humans; D016756:Immunoglobulins, Intravenous / Q000008:administration & dosage / Q000627:therapeutic use*; D008168:Lung / Q000473:pathology; D018442:Lymphoma, B-Cell, Marginal Zone / Q000150:complications* / Q000175:diagnosis / Q000188:drug therapy*; D011239:Prednisolone / Q000627:therapeutic use; D012074:Remission Induction; D016896:Treatment Outcome
D016756:Immunoglobulins, Intravenous; D011239:Prednisolone