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Kindler, O; Karastaneva, A; Seidel, MG.
SIC-reg.org: Management guidelines and registry study for severe immune cytopenias
MONATSSCHR KINDERH. 2017; 165(9): 772-779.
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Autor/innen der Med Uni Graz:
Karastaneva Anna
Seidel Markus
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Abstract:
Autoimmune manifestations belong to the clinical spectrum of primary immune deficiencies and dysregulation disorders (PID). Immune cytopenia occurs most frequently in B or T cell deficiencies and patients with an underlying PID have at least a 120-fold increased risk to develop cytopenia as compared to the general population. In a pediatric cohort of chronic immune thrombocytopenia (ITP), many other or additional diagnoses (e.g. CTLA4 haploinsufficiency, systemic lupus erythematosus and Fanconi anemia) were identified in > 30% of patients, and responses to various standard treatment approaches for ITP were highly variable, suggesting different underlying mechanisms. The awareness and the diagnostic power for underlying disorders need to be increased to improve the clinical management of patients with severe immune cytopenia. The proposed registry study aims to assist the immediate care at the clinical level and enable translational research and risk stratification using biomarkers. Panels for the diagnostic work-up will include hematological and immunological parameters and enable the exclusion of many differential diagnoses. Biomarker analyses should allow early management stratification according to patterns reminiscent of known cytopenia-related PID even if a molecular diagnosis is not yet available or inconclusive. Treatment guidelines will be derived from international standard recommendations and regularly complemented with data on newly available approaches. The natural course of the disease will be monitored. Data sets will be compatible with similar national pediatric and adult guidelines or registries. A website will provide essential information for all participants (www.SIC-reg.org).

Find related publications in this database (Keywords)
Immune thrombocytopenia
Anemia, hemolytic, autoimmune
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