Diagnosis and management of autoimmune cytopenias in childhood.
The diagnosis and management of children with autoimmune cytopenias can be challenging. 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 comorbid immunodeficiency, malignancy, rheumatologic condition, or lymphoproliferative disorder. Treatment options generally consist of nonspecific immune suppression or modulation. This nonspecific approach is changing as recent insights into disease biology have led to targeted therapies, including the use of thrombopoietin mimetics in ITP and sirolimus for cytopenias associated with autoimmune lymphoproliferative syndrome.
Pediatric Hematology, Children's Hospital of Philadelphia, University of Pennsylvania School of Medicine, 3501 Civic Center Boulevard, Philadelphia, PA 19104, USA. Electronic address: teacheyd@email.chop.edu.
David T
DT
Teachey
Michele P
MP
Lambert
D000744:Anemia, Hemolytic, Autoimmune / Q000175:diagnosis* / Q000628:therapy; D002648:Child; D006801:Humans; D007155:Immunologic Factors / Q000627:therapeutic use*; D007167:Immunotherapy / Q000379:methods*; D009503:Neutropenia / Q000175:diagnosis* / Q000628:therapy; D013921:Thrombocytopenia / Q000175:diagnosis* / Q000628:therapy
D007155:Immunologic Factors
Autoimmune hemolytic anemia; Autoimmune lymphoproliferative syndrome; Autoimmune neutropenia; Evans syndrome; Immune thrombocytopenia