Title:Mouse Models of Autoimmune Diseases: Immune Thrombocytopenia
Volume: 21
Issue: 18
Author(s): Anton Neschadim and Donald R. Branch
Affiliation:
Keywords:
Mouse models of immune thrombocytopenia, ITP, autoimmune disease, platelets, IVIg.
Abstract: Immune thrombocytopenia or ITP is a debilitating and life-threatening disorder affecting more than 4 in
every 10, 000 adults annually. Following a basic understanding of the immunopathology underlying ITP, namely
that production of anti-platelet antibodies results in accelerated platelet clearance and thrombocytopenia, animal
models of ITP were quickly developed. Rodent models that develop ITP spontaneously or by passive transfer of
anti-platelet sera or antibodies have become instrumental in investigating the mechanisms responsible for the
breakdown of tolerance in human ITP, understanding the immunopathology that underlies the progression of platelet
destruction, elucidating the mechanism(s) of therapeutic amelioration of the ITP, and driving the development of
new therapeutic modalities. This review aims to capture the development history and methodology of currently
available ITP disease models, and review their advantages and limitations in the study of various aspects of ITP. We also review how
closely the various ITP models reflect the pathobiology of human ITP and their usefulness in advancing the development of new therapeutics,
which are particularly needed to address the unmet need of patients who are refractory to the currently available repertoire of interventions.
