Intersecting paths between autoimmunity and bone marrow fibrosis: the case of autoimmune myelofibrosis

Abstract

Bone marrow fibrosis is frequently observed in patients with haematological malignancies including myeloproliferative neoplasm (MPNs). The most common cause of BMF is the BCR-ABL-negative MPN primary myelofibrosis (PMF) however, BMF may also be caused by a variety of non-neoplastic disorders such as infections, endocrine diseases and autoimmune diseases (ADs). Autoimmune myelofibrosis (AIMF) is a type of BMF associated with either a fully blown AD (secondary AIMF) or serological signs of autoimmunity without an overt AD (primary AIMF). Although isolated case reports and case series have been published, AIMF remains a poorly recognized disorder. Therefore, we performed a scoping review and critically appraised the literature on AIMF pathogenesis, diagnosis and treatment with a particular focus on similarities and differences between AIMF and PMF.

Keywords: Autoimmune diseases; Autoimmune myelofibosis; Bone marrow fibrosis; Intravenous immunoglobulins; Primary autoimmune myelofibosis; Primary myelofibrosis; Rituximab; Ruxolitinib; Secondary autoimmune myelofibosis.