Autoimmune Myelofibrosis in Sjögren's Syndrome: Report of a Case.

Publisher: International Scientific Information, Inc Country of Publication: United States NLM ID: 101489566 Publication Model: Electronic Cited Medium: Internet ISSN: 1941-5923 (Electronic) Linking ISSN: 19415923 NLM ISO Abbreviation: Am J Case Rep Subsets: MEDLINE

Publication: <2014- > : Smithtown, NY : International Scientific Information, Inc.
Original Publication: Albertson, NY : International Scientific Literature, Inc.

Autoimmune Diseases*/diagnosis ; Primary Myelofibrosis*/complications ; Primary Myelofibrosis*/diagnosis ; Sjogren's Syndrome*/complications ; Sjogren's Syndrome*/diagnosis ; Thrombocytopenia*; Bone Marrow ; Humans ; Male ; Middle Aged

BACKGROUND Autoimmune myelofibrosis (AMF) is a rare clinicopathologic entity of bone marrow fibrosis that occurs in association with autoimmune disorders. Steroids are very effective for treatment of AMF and the disease has a good prognosis and should be distinguished from primary myelofibrosis. CASE REPORT A 49-year-old man with bleeding and petechial hemorrhage of the extremities presented to our institution. His platelet count was 1×10⁹/L. Bone marrow aspiration revealed a dry tap, and bone marrow biopsy confirmed small lymphocyte infiltration and increased reticular fibers, consistent with immune thrombocytopenia. Testing for mutations in JAK2, MPL, and CALR was negative. Because the patient had a history of Raynaud's phenomenon, he was suspected to have collagen disease. Anti-Sjögren's-syndrome-related antigen-A antibody testing, Schirmer's test, and fluorescein staining all came back positive, which led to a diagnosis of Sjögren's syndrome. Given the bone marrow findings, the patient also was diagnosed with AMF. Treatment with steroids resulted in an immediate improvement in his platelet count. CONCLUSIONS In the present case, treatment with steroids resulted in prompt improvement in platelet counts and subsequent marrow biopsy showed MF-0 reticulin fibrosis. Bone marrow fibrosis rarely is seen in association with autoimmune disease, and its significance and mechanism are still to be determined.

Am J Hematol. 2014 Sep;89(9):915-25. (PMID: 25124313)
Rheumatology (Oxford). 1999 Apr;38(4):370-1. (PMID: 10378716)
Medicine (Baltimore). 1994 May;73(3):145-52. (PMID: 8190037)
Am J Hematol. 2003 Jan;72(1):8-12. (PMID: 12508261)
Leuk Lymphoma. 2004 Mar;45(3):561-6. (PMID: 15160919)
Acta Haematol. 2017;138(3):129-137. (PMID: 28866671)
Intern Med. 2014;53(7):783-7. (PMID: 24694497)
Mol Clin Oncol. 2016 Dec;5(6):789-791. (PMID: 28105358)
Hum Pathol. 2014 Nov;45(11):2183-91. (PMID: 25282037)
Eur J Intern Med. 2006 Mar;17(2):136-7. (PMID: 16490694)
Clin Adv Hematol Oncol. 2018 Sep;16(9):619-626. (PMID: 30256778)
Am J Clin Pathol. 2001 Aug;116(2):211-6. (PMID: 11488067)
Indian J Hematol Blood Transfus. 2016 Sep;32(3):368-73. (PMID: 27429532)
Am J Hematol. 2006 Jan;81(1):51-8. (PMID: 16369977)

Date Created: 20200921 Date Completed: 20210514 Latest Revision: 20210514

20231215

PMC7505477

10.12659/AJCR.924983

32956337