impact factor, citescore
logo
 

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


1, 2, 3, 4, 5, 6, 7, 8, 9

 

  1. Department of Rheumatology and Immunology, The Second Hospital of Shanxi Medical University, Taiyuan, China.
  2. Department of Rheumatology and Immunology, The Second Hospital of Shanxi Medical University, Taiyuan, China.
  3. Department of Rheumatology and Immunology, The Second Hospital of Shanxi Medical University, Taiyuan, China.
  4. Department of Rheumatology and Immunology, The Second Hospital of Shanxi Medical University, Taiyuan, China.
  5. Department of Rheumatology and Immunology, The Second Hospital of Shanxi Medical University, Taiyuan, China.
  6. Department of Haematology, The Second Hospital of Shanxi Medical University, Taiyuan, China.
  7. Department of Haematology, The Second Hospital of Shanxi Medical University, Taiyuan, China.
  8. Pathology, Brigham and Women’s Hospital, Harvard Medical School, Boston, USA.
  9. 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

Free to view
(click on article PDF icon to read the article)

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.

Rheumatology Article