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

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. CAS...

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Published in:The American journal of case reports 2020-09, Vol.21, p.e924983-e924983
Main Authors: Kakiuchi, Seiji, Takagi, Ikumi, Akiyama, Hiroaki, Matsuba, Hiroyuki, Rikitake, Junpei, Kajimoto, Kazuyoshi, Hayashi, Yoshitake, Iwata, Nobuko
Format: Article
Language:English
Subjects:
Artilces
Autoimmune Diseases - diagnosis
Bone Marrow
Humans
Male
Middle Aged
Primary Myelofibrosis - complications
Primary Myelofibrosis - diagnosis
Sjogren's Syndrome - complications
Sjogren's Syndrome - diagnosis
Thrombocytopenia
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Summary: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.
ISSN:1941-5923
1941-5923
DOI:10.12659/AJCR.924983