A Case Report of an Atypical Presentation of IgG4-Related Disease and Idiopathic CD4 Lymphocytopenia
Joint Authors
Zanoli, Luca
Rapisarda, Francesco
Castellino, Pietro
Portale, Grazia
Scuto, Salvo
Source
Issue
Vol. 2015, Issue 2015 (31 Dec. 2015), pp.1-5, 5 p.
Publisher
Hindawi Publishing Corporation
Publication Date
2015-09-28
Country of Publication
Egypt
No. of Pages
5
Main Subjects
Abstract EN
The IgG4-related disease is a fibroinflammatory disease characterized by tumefactive lesions, a dense lymphoplasmacytic infiltrate rich in IgG4-positive plasma cells, storiform fibrosis, and, often but not always, elevated serum levels of IgG4.
Idiopathic CD4 lymphocytopenia is a heterogenic and rare syndrome characterized by the detection of a persistent absolute CD4 T cells count <300 cells/mm3 (or <20% of total T cells) in more than one occasion and no evidence of HIV infection in absence of immunodeficiency or therapy associated with depressed levels of CD4 T cells.
We report the case of a 50-year-old man with a multiorgan IgG4-related disease presenting in a temporal association with a profound and symptomatic idiopathic CD4 lymphocytopenia.
Both clinical pictures improved after steroid treatment.
Idiopathic CD4 lymphocytopenia has been associated with a number of autoimmune conditions but, to the best of our knowledge, this is the first case in which an association with the IgG4-related disease is reported.
American Psychological Association (APA)
Rapisarda, Francesco& Zanoli, Luca& Portale, Grazia& Scuto, Salvo& Castellino, Pietro. 2015. A Case Report of an Atypical Presentation of IgG4-Related Disease and Idiopathic CD4 Lymphocytopenia. Case Reports in Medicine،Vol. 2015, no. 2015, pp.1-5.
https://search.emarefa.net/detail/BIM-1058859
Modern Language Association (MLA)
Rapisarda, Francesco…[et al.]. A Case Report of an Atypical Presentation of IgG4-Related Disease and Idiopathic CD4 Lymphocytopenia. Case Reports in Medicine No. 2015 (2015), pp.1-5.
https://search.emarefa.net/detail/BIM-1058859
American Medical Association (AMA)
Rapisarda, Francesco& Zanoli, Luca& Portale, Grazia& Scuto, Salvo& Castellino, Pietro. A Case Report of an Atypical Presentation of IgG4-Related Disease and Idiopathic CD4 Lymphocytopenia. Case Reports in Medicine. 2015. Vol. 2015, no. 2015, pp.1-5.
https://search.emarefa.net/detail/BIM-1058859
Data Type
Journal Articles
Language
English
Notes
Includes bibliographical references
Record ID
BIM-1058859