Primary myelofibrosis but not autoimmune myelofibrosis accompanied by Sj ögren's syndrome and primary biliary cirrhosis in a patient with trisomy 8 mosaic: a case report and literature review.

Primary myelofibrosis but not autoimmune myelofibrosis accompanied by Sjögren's syndrome and primary biliary cirrhosis in a patient with trisomy 8 mosaic: a case report and literature review. Clin Exp Rheumatol. 2018 May 10; Authors: Lu C, Wu X, Wen H, Gao H, Wang C, Yang B, Liang Z, Gao C, Li X Abstract Bone marrow fibrosis has been found to be associated with autoimmune disorders, and autoimmune myelofibrosis (AIMF) has been defined. Primary myelofibrosis (PMF), a clonal myeloproliferative disorder, should be distinguished from AIMF which has a good response to steroids, as the former has a high mortality and very bad response to conventional treatment. This case report describes a rare case of PMF accompanied with Sjögren's syndrome (SJS) and primary biliary cirrhosis (PBC), in a patient with trisomy 8 mosaic. Careful clinical assessment, gene mutation screening, and bone marrow evaluation can lead to an accurate diagnosis. PMID: 29745886 [PubMed - as supplied by publisher]
Source: Clinical and Experimental Rheumatology - Category: Rheumatology Tags: Clin Exp Rheumatol Source Type: research