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Concurrent Autoimmune Neutropenia and Idiopathic Thrombocytopenic Purpura Associated with IgG4-related Diease.

Authors
  • Shimazu, Yayoi1
  • Uchiyama, Tatsuki1
  • Mizumoto, Chisaki1
  • Takeoka, Tomoharu1
  • Tsuji, Masaaki1
  • Tomo, Kenjiro1
  • Takaori, Koji2
  • Sakai, Naoki3
  • Okuno, Tomoko4
  • Ohno, Tatsuharu1
  • 1 Department of Hematology and Immunology, Ohtsu Red Cross Hospital, Japan. , (Japan)
  • 2 Department of Nephrology, Ohtsu Red Cross Hospital, Japan. , (Japan)
  • 3 Department of Respiratory Medicine, Ohtsu Red Cross Hospital, Japan. , (Japan)
  • 4 Department of Clinical Pathology, Ohtsu Red Cross Hospital, Japan. , (Japan)
Type
Published Article
Journal
Internal medicine (Tokyo, Japan)
Publication Date
Feb 28, 2018
Identifiers
DOI: 10.2169/internalmedicine.0190-17
PMID: 29491289
Source
Medline
Keywords
License
Unknown

Abstract

IgG4-related disease (IgG4RD) is a multi-organ disorder characterized by an elevated serum IgG4 level and IgG4-positive plasma cell infiltration of the affected organs, accompanied by tissue fibrosis and sclerosis. Although it can affect any organ, to our knowledge, no cases involving concurrent autoimmune neutropenia and thrombocytopenia have been reported. A 62-year-old man visited our hospital and was diagnosed with IgG4RD accompanied by interstitial pneumonitis, lymphadenopathy, and interstitial nephritis. During his clinical course, he developed autoimmune neutropenia and idiopathic thrombocytopenic purpura. Our case, invoving multiple hematological abnormalities, might help deepen our understanding of the pathophysiology of IgG4RD.

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