Abstract
Immune thrombocytopenia (ITP) is an autoimmune disease that is characterized by a
significant reduction in the number of circulating platelets and frequently associated
with bleeding. Although the pathogenesis of ITP is still not completely elucidated,
it is largely recognized that the low platelet count observed in ITP patients is due
to multiple alterations of the immune system leading to increased platelet destruction
as well as impaired thrombopoiesis. The clinical manifestations and patients' response
to different treatments are very heterogeneous suggesting that ITP is a group of disorders
sharing common characteristics, namely, loss of immune tolerance toward platelet (and
megakaryocyte) antigens and dysfunctional primary hemostasis. Management of ITP is
challenging and requires intensive communication between patients and caregivers.
The decision to initiate treatment should be based on the platelet count level, age
of the patient, bleeding manifestation, and other factors that influence the bleeding
risk in individual patients. In this review, we present recent data on the mechanisms
that lead to platelet destruction in ITP with a particular focus on current findings
concerning alterations of thrombopoiesis. In addition, we give an insight into the
efficacy and safety of current therapies and management of ITP bleeding emergencies.
Keywords
platelet immunology - immune thrombocytopenia - autoantibodies - autoimmune diseases
