title: Myelodysplasia and malignancy-associated vasculitis.
abstract: While the link between malignancy and vasculitis has been known for some time, the association of vasculitis and myelodysplastic syndrome (MDS) has only recently been reported. This article reviews the most current and landmark publications regarding MDS, as well as malignancy-associated vasculitis. We include theories of paraneoplastic associations, immune pathogenesis including an associated cytokine transcriptional factor (interferon regulatory factor-1 [IFN-1]), and the relationship to treatment. Key clinical features that suggest underlying malignancy in patients with vasculitis are highlighted. Although the association between vasculitis and malignancy is rare, leukocytoclastic vasculitis is the most common vasculitis associated with MDS, hematologic malignancies as well as solid tumors. We review several articles that demonstrate a paraneoplastic association between vasculitis and various malignancies, but overall, the connection is still unclear and not well defined. Certain features that suggest a true paraneoplastic association are outlined. Further studies are needed to advance our understanding of this complex topic.
authors: Department of Medicine and Rheumatology, James A. Haley VA and University of South Florida, 13000 Bruce B. Downs Boulevard (111K), Tampa, FL 33612, USA. aahga@health.usf.edu
authors: Amir
authors: A
authors: Agha
authors: Helen
authors: H
authors: Bateman
authors: Ashley
authors: A
authors: Sterrett
authors: Joanne
authors: J
authors: Valeriano-Marcet
mesh_terms: D019337:Hematologic Neoplasms / Q000150:complications / Q000378:metabolism; D006801:Humans; D050856:Interferon Regulatory Factor-1 / Q000378:metabolism; D009190:Myelodysplastic Syndromes / Q000150:complications* / Q000378:metabolism; D009369:Neoplasms / Q000150:complications* / Q000378:metabolism; D010257:Paraneoplastic Syndromes / Q000150:complications* / Q000378:metabolism; D014657:Vasculitis / Q000150:complications* / Q000378:metabolism; D018366:Vasculitis, Leukocytoclastic, Cutaneous / Q000150:complications / Q000378:metabolism
chemical_list: C494257:IRF1 protein, human; D050856:Interferon Regulatory Factor-1