Clonality of Lymphocytic Interstitial Pneumonia in Common Variable Immunodeficiency.
- Author:
Kye Wool KANG
1
;
Young Sub LEE
;
Hwang Min KIM
;
Byung Il YAE
;
Tae Heon KIM
;
Soon Hee JUNG
;
Dong Jin KIM
;
Kyung A LEE
Author Information
1. Department of Pediatrics, College of Wonju Medicine, Younsei University, Wonju, Korea. kkw_us@hanmail.net
- Publication Type:Case Report
- Keywords:
Common variable immunodeficiency;
Lymphocytic interstitial pneumonia;
CDR3;
Malignant lymphoma
- MeSH:
Adolescent;
Agammaglobulinemia;
Autoimmune Diseases;
Bacterial Infections;
Biopsy;
Clone Cells;
Common Variable Immunodeficiency*;
Complementarity Determining Regions;
Female;
Humans;
Hyperplasia;
Immunoglobulin Heavy Chains;
Lip;
Lung;
Lung Diseases, Interstitial*;
Lymphocytes;
Lymphoma;
Lymphoproliferative Disorders
- From:Korean Journal of Pediatric Hematology-Oncology
2001;8(2):355-360
- CountryRepublic of Korea
- Language:Korean
-
Abstract:
Common variable immunodeficiency (CVID) is a heterogenous syndrome characterized by hypogammaglobulinemia, various immunologic abnormalities and recurrent bacterial infections. Associated immunologic abnormalities consists of various kinds of autoimmune diseases and lymphoproliferative disorders. The lymphoproliferative disorder take several forms, such as malignant lymphoma, atypical lymphoid hyperplasia, and beniegn lymphoid hyperplasia. Lymphocytic interstitial pneumonia (LIP), which is a kind of atypical lymphoid hyperplasia, develop in young age groups and has controversy on its clonality. We experienced a 14-year-old female patient with LIP and CVID. We analysed the third complementarity-determining region (CDR3) of the immunoglobulin heavy chain gene for clonality analysis. Clonality analysis of lung biopsy specimen revealed that 6 of 13 colony and 4 of 13 colony have identical sequences respectively. We speculate that one of these 2 lymphoid cell clone may develop into malignant lymphoma in the future.
