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Deprecated: Implicit conversion from float 209.6 to int loses precision in C:\Inetpub\vhosts\kidney.de\httpdocs\pget.php on line 534 Pediatr+Clin+North+Am 2013 ; 60 (6): 1489-511 Nephropedia Template TP
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Diagnosis and Management of Autoimmune Cytopenias in Childhood #MMPMID24237984
Teachey DT; Lambert MP
Pediatr Clin North Am 2013[Dec]; 60 (6): 1489-511 PMID24237984show ga
The diagnosis and management of children with autoimmune cytopenias can be challenging, as this diagnostic category encompasses a number of heterogeneous but closely related conditions. Children can present with immune-mediated destruction of a single cell lineage or multiple cell lineages, including platelets (immune thrombocytopenia, ITP), erythrocytes (autoimmune hemolytic anemia), and neutrophils (autoimmune neutropenia). Immune-mediated destruction can be primary or secondary to a co-morbid immunodeficiency, malignancy, rheumatologic condition, or lymphoproliferative disorder. Despite the heterogeneity, treatment options are similar and generally consist of non-specific immune suppression or modulation. Fortunately, this non-specific approach is changing as recent insights into disease biology have led to targeted therapies for certain patients, including the use of thrombopoietin mimetics in ITP and sirolimus for cytopenias associated with autoimmune lymphoproliferative syndrome (ALPS).