Inflammatory and Immune Disorders Associated with Myelodysplastic Syndromes

Abstract

Systemic auto-inflammatory or autoimmune diseases (SIADs) develop in up to a quarter of patients with myelodysplastic syndromes (MDS) or chronic myelomonocytic leukemia (CMML). With or without the occurrence of SIADs, the distribution of MDS subtypes and the international or CMML-specific prognostic scoring systems have been similar between MDS/CMML patients. Moreover, various SIADs have been described in association with MDS, ranging from limited clinical manifestations to systemic diseases affecting multiple organs. Defined clinical entities including systemic vasculitis, connective tissue diseases, inflammatory arthritis and neutrophilic diseases are frequently reported; however, unclassified or isolated organ impairment can also be seen. Although the presence of SIADs does not impact the overall survival nor disease progression to acute myeloid leukemia, they can help with avoiding steroid dependence and make associated adverse events of immunosuppressive drugs challenging. While therapies using steroids and immunosuppressive treatment remain the backbone of first-line treatment, increasing evidence suggests that MDS specific therapy (hypomethylating agents) and sparing steroids may be effective in treating such complications based on their immunomodulatory effect. The aim of this review was to analyze the epidemiological, pathophysiological, clinical and therapeutic factors of systemic inflammatory and immune disorders associated with MDS.

Pretransplant Systemic Lipidomic Profiles in Allogeneic Stem Cell Transplant Recipients

“…MDS is associated with inflammatory complications [ 65 ], but despite this we did not find any significant association between pretransplant signs of inflammation and MDS/AML-MDS. This observation suggests that the risk of posttransplant acute GVHD/nonrelapse mortality associated with pretransplant inflammation [ 12 , 13 ] is similar for patients with and without MDS/MDS-AML.…”

“…Previous studies, as well as a large meta-analysis, have shown that pretransplant signs of inflammation are associated with decreased survival and increased acute GVHD [ 12 , 13 ]; an increased early mortality for this group is therefore not unexpected. Furthermore, even though inflammatory/immune complications are increased among MDS patients [ 65 ], we could not detect any difference in CRP levels between the patients with MDS/MDS-AML and the other patients. The frequency of GVHD (together with the risk of relapse) is regarded as a major challenge in allotransplanted MDS patients [ 81 , 87 ], and recent studies suggest that intensified GVHD prophylaxis should be considered for MDS patients [ 87 , 88 ].…”

“…When analyzing the total serum concentrations of various lipid subclasses we detected a subset of patients with higher age, higher frequency of MDS or MDS-AML and more frequent signs of inflammation together with lower BMI and reduced levels of lipids belonging to several lipid subclasses. Signs of inflammation are associated with an adverse prognosis after allogeneic stem cell transplantation [ 13 ], and signs of inflammation are also common in MDS [ 65 ]. Differences in inflammatory regulation may also contribute to the advantage of using younger matched unrelated donors instead of matched sibling donors in elderly AML patients [ 66 , 67 ].…”