Patients presenting with bone marrow fibrosis not accompanied by well-established autoimmune diseases, such as systemic lupus erythematosus, or malignant diseases, are considered to have primary autoimmune myelofibrosis (AIMF). Primary AIMF has been reported to follow a benign course and responds well to treatment with immunosuppressive agents. Immune thrombocytopenia (ITP) is also an autoimmune disorder characterized by antiplatelet-antibody-mediated thrombocytopenia in the absence of other causes of thrombocytopenia. We herein present a rare case of a female patient who was diagnosed with primary AIMF. The patient presented with severe thrombocytopenia, which was initially misdiagnosed as ITP. The symptoms of the disease resolved completely following steroid treatment. After withdrawal of the treatment at 1 year from the diagnosis, the bone marrow examination showed no evidence of bone marrow fibrosis or other abnormalities. To date, the patient has been followed up for 2 years without evidence of disease.
Keywords: immune thrombocytopenia; primary autoimmune myelofibrosis; steroids.