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

Abstract

Patient: Male, 49-year-old Final Diagnosis: Autoimmune myelofibrosis Symptoms: Bleeding Medication:— Clinical Procedure: Bone marrow biopsy Specialty: Hematology Objective: Rare disease 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 1×10 9 /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.

TAFRO syndrome is associated with anti-SSA/Ro60 antibodies, in contrast to idiopathic castleman disease

“…Some cases of SS with protein-losing enteropathy have pleural ascites and generalized edema 31 ; renal dysfunction and thrombocytopenia are observed in 4% and 8.1% of SS patients, respectively 32 . Moreover, myelofibrosis has been reported in a few cases 33 . In the present cases, the patients did not have symptoms of dryness, but the objective assessment of dryness was positive in three of six patients.…”