Korean J Pediatr Hematol Oncol.  2001 Oct;8(2):355-360.

Clonality of Lymphocytic Interstitial Pneumonia in Common Variable Immunodeficiency

Affiliations
  • 1Department of Pediatrics, College of Wonju Medicine, Younsei University, Wonju, Korea. kkw_us@hanmail.net
  • 2Department of Biochemistry, College of Wonju Medicine, Younsei University, Wonju, Korea.
  • 3Department of Pathology, College of Wonju Medicine, Younsei University, Wonju, Korea.
  • 4Department of Radiology, College of Wonju Medicine, Younsei University, Wonju, Korea.
  • 5Department of Clinicopathology, College of Medicine, Sungkyunkwan University, Samsung Hospital, Seoul, Korea.

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.

Keyword

Common variable immunodeficiency; Lymphocytic interstitial pneumonia; CDR3; Malignant lymphoma

MeSH Terms

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
Complementarity Determining Regions
Immunoglobulin Heavy Chains
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