DOES OUTCOME/SURVIVAL OF PATIENTS WITH MYELODYSPLASTIC SYNDROMES SHOULD BE PREDICTED BY REDUCED LEVELS OF ADAMTS-13? RESULTS FROM A PILOT STUDY.

Abstract: Introduction Von Willebrand factor (vWF) cleaving protease ADAMTS-13 has a key role for maintaining normal size of vWF. A deficiency or dysfunction of vWF cleaving protease is associated with ultra large vWF multimers and thrombotic microangiopathy (TMA). Patients with cancers have reduced levels of vWF cleaving protease. In this pilot study we have evaluated whether or not deficiencies of ADAMTS-13 were present in myelodysplastic syndromes (MDS). Moreover, we assessed if a reduction in basal levels of ADAMTS-13 may play a role in the prognosis of MDS. Patients and methods We measured and compared the levels of vWF cleaving protease ADAMTS-13 in 100 MDS patients and in 35 healthy controls. Patients were divided in two groups according to IPSS: group I consisting of 44 low risk MDS patients, group II of 56 high-risk MDS patients. Patients with high risk and low risk MDS presented significantly lower levels of ADAMTS-13 than controls (p Results We found that reduced levels of ADAMTS-13 have a relationship with overall survival (OS) (p Conclusions ADAMTS-13 in MDS might represent a surrogate marker of prognosis, response to therapy, or disease progression. Further studies are needed.