Case Report
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
C. Lu1, X. Wu2, H. Wen3, H. Gao4, C. Wang5, B. Yang6, Z. Liang7, C. Gao8, X. Li9
- Department of Rheumatology and Immunology, The Second Hospital of Shanxi Medical University, Taiyuan, China.
- Department of Rheumatology and Immunology, The Second Hospital of Shanxi Medical University, Taiyuan, China.
- Department of Rheumatology and Immunology, The Second Hospital of Shanxi Medical University, Taiyuan, China.
- Department of Rheumatology and Immunology, The Second Hospital of Shanxi Medical University, Taiyuan, China.
- Department of Rheumatology and Immunology, The Second Hospital of Shanxi Medical University, Taiyuan, China.
- Department of Haematology, The Second Hospital of Shanxi Medical University, Taiyuan, China.
- Department of Haematology, The Second Hospital of Shanxi Medical University, Taiyuan, China.
- Pathology, Brigham and Women’s Hospital, Harvard Medical School, Boston, USA.
- Department of Rheumatology and Immunology, The Second Hospital of Shanxi Medical University, Taiyuan, China. 13753139859@163.com
CER11040
2018 Vol.36, N°3 ,Suppl.112
PI 0234, PF 0236
Case Report
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PMID: 29745886 [PubMed]
Received: 11/12/2017
Accepted : 05/03/2018
In Press: 10/05/2018
Published: 14/08/2018
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.
