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Myelodysplastic Syndromes and autoimmune disorders: Cause or consequence?

PubMed Abstract: 
Original Publication Date: 
Thursday, June 12, 2014

This article reviews Myelodysplastic Syndromes (MDS) and the association with autoimmune disorders (AD). AD are frequently found in MDS patients, ranging from 10-30%. Patients generally are diagnosed with MDS around the time of symptomatic AD. Those patients with AD have a higher risk to develop MDS or acute myeloid leukemia (AML) than the general population – diseases including rheumatoid arthritis, Sjogren syndrome, lupus, seronegative arthritis, panarteritis nodosa, autoimmune hemolysis, and pernicious anemia.

Those with both MDS and AD display clinical manifestations of AD 10-30% of the time – most frequently being vasculitis, seronegative polyarthritis, and skin lesions. Several studies illustrate this statistically significant increase in AD manifestations. The majority of patients with both MDS and AD are male (up to 70%) and older in age (mean 78-83 years). There are no cytogenetic abnormalities that are associated with both MDS and AD – other than trisomy 8 in Behcet’s disease. It is unknown if AD have an impact on overall survival in MDS patients.

The most common AD clinical manifestations in MDS patients involve: vasculitis, Behcet’s disease, inflammatory arthritis, and neutrophilic dermatosis. Vasculitis is most common in refractory anemia with excess blasts (RAEB) and chronic myelomonocytic leukemia (CMML). Sweet’s syndrome, a subtype of neutrophilic dermatosis, is associated with both AML and MDS, 40% and 15% respectively. Relapsing polychondritis, glomerulonephritis, Crohn’s disease, and alveolar proteinosis are other clinical autoimmune phenomena reported in case reports.

In addition to clinical manifestations, there is an increased number of abnormal immune laboratory findings in MDS patients, such as polyclonal hypergammaglobulinemia , antinuclear antibodies, and inflammatory cytokines (IL-6, TNF-α, IF-γ). These complex auto reactive immune responses may further induce cytopenias in MDS patients. For instance, deregulated T cell immunity which leads to downstream inflammatory responses can induce bone marrow failure due to apoptosis. Due to increased production of these cytokines and impaired cellular immunity, these are the current areas of research for potential drug targets. For now, MDS patients with AD are generally treated with immunosuppressive therapies (such as immunomodulatory agents (ImiDs), antithymocyte globulin (ATG), and alemtuzumab) due to the autoimmune component.

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