The burden of autoimmunity in myelodysplastic syndromes

Abstract

The clinical history of patients with myelodysplastic syndromes (MDS) is characterised by bone marrow insufficiency as well as by the possible evolution into acute leukaemia. However a number of reports highlight the frequent occurrence of autoimmune manifestations involving different sites and organs. The present review will first describe the clinical pictures most often observed in MDS patients. The actual burden of autoimmunity will be then addressed by focusing on the few available registry studies. Finally, the potential collateral impact of specific treatments for MDS on the evolution of autoimmune disorders will be considered.

Autoimmune Complications in Hematologic Neoplasms

“…As regards MDS, AIHA mainly occur in the low-risk setting [37], developing one to many years after MDS diagnosis [51]. ITP has been reported in few, isolated cases, often in association with other autoimmune manifestations [50,94]. Also PRCA has been mainly described in the context of low-risk MDS, showing a favorable response to immunosuppressive treatment [63].…”

“…AID are described in up to 20-30% of MDS patients [50,68]. Two thirds of AIDs develop months/years after MDS diagnosis, and mainly involve vasculitis [94].…”

“…Moreover, MDS-associated Behçet's-like syndrome has a delayed onset, greater gastrointestinal and oral involvement and reduced ocular manifestations than the idiopathic form [70,71]. Other frequent associations are relapsing polychondritis and SLE, whilst SS, RA, polymyalgia rheumatica, uveitis, pulmonary proteinosis, thyroiditis, and myositis are sporadically reported [50,68,94]. Neutrophilic dermatosis, both isolated and in the context of relapsing polychondritis, may be associated with the 5q-syndrome, worsening MDS prognosis [72].…”

“…The impact of AID on MDS prognosis is controversial. Some large retrospective studies suggest a reduced overall survival for MDS patients with AID, especially for cryoglobulinemic vasculitis, due to higher infectious rate [50]. Conversely, other studies reported no impact on survival, even if increased cardiovascular comorbidity has been reported in MDS with AID [69,70,73].…”

Vasculitis in Myelodysplastic Syndrome and Chronic Myelomonocytic Leukemia: A Report of Two Cases

“…Chronic myelomonocytic leukemia (CMML), initially categorized within MDS, is currently classified as myelodysplastic/myeloproliferative neoplasm [1,3]. There is a correlation between MDS/CMML and the presence of a broad spectrum of autoimmune manifestations (AIM) [4][5][6][7][8][9]. Patients with autoimmune diseases have an increased risk for the development of MDS with an odds ratio of 1.5 to 3.5, and clinically apparent AIM occur in 5-35% of the MDS, while up to 60% of MDS patients may have serological signs of autoimmunity [8,9].…”