Abstract:
:Thrombotic microangiopathy (TMA)/thrombotic thrombocytopenic purpura (TTP) is a rare but potentially lethal condition requiring rapid recognition, diagnosis, and initiation of therapy. We experienced a case of a 61-year-old woman with primary Sjögren's syndrome (pSS) complicated with severe renal TMA/TTP following IgM monoclonal gammopathy of undetermined significance (MGUS). She was admitted to our hospital for further evaluation of hypergammaglobulinema, acute renal failure, and severe thrombocytopenia. She had been diagnosed with pSS 13 years prior to admission. Histological examination of her kidney revealed fibrin thrombi in the glomeruli and arterioles, a finding that is consistent with TMA/TTP. The patient was subsequently treated with plasma exchange, which resulted in a successful outcome without any complications. This rare case suggests that it is important to make a therapeutic decision based on appropriate and prompt pathological diagnosis.
journal_name
Rheumatol Intjournal_title
Rheumatology internationalauthors
Koga T,Yamasaki S,Nakamura H,Kawakami A,Furusu A,Taguchi T,Eguchi Kdoi
10.1007/s00296-010-1569-0subject
Has Abstractpub_date
2013-01-01 00:00:00pages
227-30issue
1eissn
0172-8172issn
1437-160Xjournal_volume
33pub_type
杂志文章abstract::In spite of differences in etiology, RA and OA lead to astonishingly similar synovitic alterations. Fibroblastic transformation of the synovial membrane and an increase in monocytes constitute a rare but highly characteristic feature of RA. Monocytes synthesize factor (F) XIII, implying that FXIII (a and s) in synovia...
journal_title:Rheumatology international
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pub_type: 临床试验,杂志文章
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pub_type: 杂志文章,随机对照试验
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