Aim: To evaluate the incidence and outcome of secondary immune thrombocytopenia (ITP) in a large cohort of paediatric Spanish patients.
Methods: A retrospective observational study was conducted in two paediatricUniversity hospitals in Spain between 2009 and 2019, which included children from 4 months to 18 years old diagnosed with ITP. Data were recorded from clinical charts: gender, age at diagnosis, coexisting condition and associated characteristics, outcome and treatment.Results: Secondary ITP was diagnosed in 87 out of 442 patients (19.6%). Postimmunisation ITP was seen in younger children. The onset of secondary ITP to autoimmune diseases (AD) and immunodeficiencies (ID) was at an older age and had more tendency to be insidious, and platelet level was higher than primary ITP. Mean time from ITP onset to AD diseases or ID diagnosis was 1.2 and 2.6 years, respectively. Whereas the cumulative incidence of remission was significantly higher in postimmunisation and post-viral infection (compared with primary ITP patients), it was worse in AD and ID patients.Conclusions: Identification of secondary ITP is important as it predicts outcome. Most of them are diagnosed at ITP onset, but AD diseases and ID should be ruled out periodically as they are usually identified later.
It is not clear if platelet responses are sustained after thrombopoietin receptor agonist (ar‐TPO) withdrawal in paediatric patients. A multicentre retrospective observational study was performed in children with chronic immune thrombopenia (cITP) to describe ar‐TPO tapering and withdrawal in patients who had achieved a sustained complete response to ar‐TPOs. Ten patients (eltrombopag n = 6, romiplostim n = 4) were included. Treatment withdrawal was performed after a mean tapering time of 7.6 months. Two patients relapsed (median follow‐up time of 24 months). Slow tapering and withdrawal of ar‐TPOs can be safely performed in cITP paediatric patients after achieving a sustained complete response.
Idiopathic purpura fulminans (IPF) is a rare but severe prothrombotic
coagulation disorder. Acquired IPF is usually due to a cross-reactivity
against virus and anticoagulant proteins; Protein C and less commonly
Protein S. This molecular mimicry generates antibodies against
anticoagulant proteins. Most used treatments are fresh frozen plasma
(FFP) and heparin. In refractory cases immunoglobulins, corticosteroids
and plasmapheresis can be used. Furthermore, debridement of necrotic
areas is sometimes needed. Skin involvement commonly appears in lower
limbs or torso, in contrast of other types of purpura fulminans were a
more acral involvement is seen. Our case describes for the first time an
IPF with facial location.
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