[Thrombosis and platelet dysfunction in myeloproliferative neoplasms]

Area of Science:

• Hematology
• Oncology
• Hemostasis

Background:

• Myeloproliferative neoplasms (MPNs) are acquired hematological malignancies primarily affecting adults.
• Morbidity and mortality in MPNs are significantly linked to hemostasis disorders, particularly thrombosis and bleeding.
• Thrombotic complications frequently occur in arterial territories and atypical locations like splanchnic veins.

Purpose of the Study:

• To review the complex pathophysiology of thrombosis in MPNs.
• To discuss the mechanisms underlying bleeding manifestations, including platelet dysfunctions and acquired von Willebrand syndrome.
• To highlight recent advances in understanding these hemostasis disorders in MPNs.

Main Methods:

• Literature review focusing on physiopathological mechanisms of thrombosis and bleeding in MPNs.
• Analysis of factors contributing to hemostasis disorders: blood cells, endothelium, and flow conditions.
• Examination of validated risk factors for thrombosis (age > 60, prior thrombotic events).

Main Results:

• Thrombosis pathophysiology involves intricate interactions between blood cells, endothelium, and hemodynamics.
• Hemorrhagic manifestations are linked to primary hemostasis alterations, such as platelet dysfunction and acquired von Willebrand syndrome.
• Current risk stratification for thrombosis relies on age and history of thrombotic events.

Conclusions:

• The pathophysiology of thrombosis and platelet dysfunction in MPNs is not fully understood.
• Further research into the underlying mechanisms is crucial for improving patient management.
• Understanding these disorders is key to addressing morbidity and mortality in MPNs.