Pediatric immune thrombocytopenia (ITP) is an acquired disorder
associated with autoimmune destruction and impairment of platelet
production in children. Some children exhibit poor or transient response
to ITP-directed treatments and are referred to as having refractory ITP
(rITP). There is currently no consensus on the definition of rITP, nor
evidence-based treatment guidelines for patients with rITP. After a
survey of pediatric ITP experts demonstrated lack of consensus on
pediatric rITP, we pursued a systematic review to examine the reported
clinical phenotypes and treatment outcomes in pediatric rITP. The search
identified 253 relevant manuscripts; following review, 11 studies
proposed a definition for pediatric rITP with no consensus amongst them.
Most definitions included sub-optimal response to medical management,
while some outlined specific platelet thresholds to define this
sub-optimal response. Common attributes identified in this study should
be used to propose a comprehensive definition, which will facilitate
outcome comparisons of future rITP studies.