Characteristics of Myelodysplastic Syndrome with Coagulation Abnormalities and Tailored Diagnosis and Treatment.

Abstract

At onset, myelodysplastic syndrome (MDS) may be complicated by coagulation and fibrinolytic abnormalities, such as disseminated intravascular coagulation (DIC), tumor lysis syndrome (TLS), infection, thromboembolism, hemophagocytic syndrome/hemophagocytic lymphohistiocytosis (HPS/HLH), hemorrhage, and hematoma formation. In these cases, the cause may be secondary. On the other hand, it is known that platelet clotting dysfunction and fibrinolysis abnormalities are seen in the background of MDS, and primary fibrinolysis abnormalities may be complicated by adverse events associated with paraneoplastic syndrome (PNS). Coagulation fibrinolysis, as a PNS associated with MDS, is known to take the pattern of either consumptive coagulation abnormality or fibrinolytic coagulation abnormality. One mechanism of coagulation and fibrinolytic abnormalities has been shown to be the immunophenotypical pathway, and aberrant cytokine production is also associated with coagulopathy in MDS. We focused on how to differentiate an MDS-associated bleeding tendency resulting from either secondary or primary causes. In order to make this differentiation, we proposed a useful flowchart for the differentiation of solidified fibrinolysis seen at the initial MDS diagnosis. Additionally, we compared and summarized the molecular pathways of the secondary and primary causes of coagulopathy. Addressing coagulation and fibrinolytic abnormalities in MDS is required to differentiate the complexity and heterogeneity of bleeding and coagulation abnormalities. This review highlights the need to distinguish between the primary (disease-intrinsic) and secondary (reactive or complication-related) causes of coagulopathy. By proposing a diagnostic flowchart tailored to evaluate these causes at initial diagnosis, this study supports individualized risk stratification and management strategies. By comparing the molecular pathways of the two causes of coagulopathy, we provide a clinical discussion of the underlying pathologies. This aligns with the principles of personalized medicine by ensuring that treatment decisions (e.g., supportive care, anticoagulation, and antifibrinolytics) are based on the patient's specific pathophysiological profile, rather than a one-size-fits-all approach.