The Centenary of Immune Thrombocytopenia—Part 2: Revising Diagnostic and Therapeutic Approach

Abstract

Primary immune thrombocytopenia (ITP) is the most common cause of thrombocytopenia in children and adolescents and can be considered as a paradigmatic model of autoimmune disease. This second part of our review describes the clinical presentation of ITP, the diagnostic approach and overviews the current therapeutic strategies. Interestingly, it suggests an algorithm useful for differential diagnosis, a crucial process to exclude secondary forms of immune thrombocytopenia (IT) and non-immune thrombocytopenia (non-IT), which require a different therapeutic management. Advances in understanding the pathogenesis led to new therapeutic targets, as thrombopoietin receptor agonists, whose role in treatment of ITP will be discussed in this work.

Secondary immune thrombocytopenia in children: Characteristics and outcome of a large cohort from two Spanish centres

“…Secondary ITP to vaccines administration was, in fact, the most common group in our series (30%), followed by those diagnosed after viral infection (EBV, CVM, VVZ and parvovirus B19) (29%). These were expected results as it is well known that the most likely ITP trigger is through viral antigen‐induced molecular mimicry and children are strongly exposed to them during infancy 6 . Patients in these groups (ITP post‐vaccination and viral infection) were characterised by a high and quick remission rate.…”

“…These were expected results as it is well known that the most likely ITP trigger is through viral antigen-induced molecular mimicry and children are strongly exposed to them during infancy. 6 Patients in these groups (ITP post-vaccination and viral infection) were characterised by a high and quick remission rate. This might help physicians decrease parental anxiety and appointments in the absence of bleeding symptomatology.…”

“…It is also important to remember that in ES, haemolysis could precede thrombocytopenia or appear after an undetermined time from ITP initial diagnosis. 3,12 The diagnosis of other AD was anecdotical, but since an association between ITP and thyroid AD has been reported, 6 they should also be ruled out periodically in chronic ITP paediatric patients. 5,15 F I G U R E 1 Mean cumulative incidence of remission in primary ITP and each group of secondary ITP.…”

“…Secondary ITP cases are a group of immune‐mediated thrombocytopenia associated with coexisting conditions as underlying autoimmune diseases (AD), immunodeficiencies (ID), viral infections, vaccine administration or drug exposure 3,5 . Secondary ITP has a complex aetiology as its various forms have different immunologic underpinnings 3,6 . The aim of this study was to evaluate the actual incidence of secondary ITP, its treatment and outcomes in a large cohort of paediatric Spanish patients.…”

Screening for Genetic Mutations for the Early Diagnosis of Common Variable Immunodeficiency in Children With Refractory Immune Thrombocytopenia: A Retrospective Data Analysis From a Tertiary Children's Center

“…A meta-analysis in 2014 found that positive ANA could be one of the predictors of CITP in children (63). A review of pathogenesis and therapy for primary ITP also emphasized that pediatric ITP tended to develop into CITP with some predictors including ANA positivity (64). In summary, positive indicators of the autoimmune disease might not be sensitive enough in differentiating CVID from primary ITP.…”