Diagnosis and management of chronic and refractory immune cytopenias in children, adolescents, and young adults
| Autor: | Seth Rotz, Ashish Kumar, Russell Ware |
|---|---|
| Rok vydání: | 2018 |
| Předmět: |
Male
0301 basic medicine Pediatrics medicine.medical_specialty Evans syndrome Adolescent Autoimmune Diseases Young Adult 03 medical and health sciences Immune system Refractory hemic and lymphatic diseases medicine Humans Medical diagnosis Young adult Child Genetic testing Cytopenia medicine.diagnostic_test business.industry Common variable immunodeficiency Hematology medicine.disease Hematologic Diseases 030104 developmental biology Oncology Pediatrics Perinatology and Child Health Female business |
| Zdroj: | Pediatric Blood & Cancer. 65:e27260 |
| ISSN: | 1545-5009 |
| DOI: | 10.1002/pbc.27260 |
| Popis: | Children, adolescents, and young adults with chronic refractory autoimmune cytopenias represent a rare but challenging group of patients, who are managed frequently by pediatric hematologists. Novel diagnostic tests and genomic discoveries are refining historical diagnoses of Evans syndrome and common variable immunodeficiency, while also elucidating the cellular and molecular basis for these disorders. Genetic characterization of chronic and refractory autoimmune cytopenias has led to targeted therapies with improved clinical outcomes and fewer off-target toxicities. In this review, we focus on the appropriate diagnostic workup, expanded genetic testing, and novel treatment opportunities that are available for these challenging patients. |
| Databáze: | OpenAIRE |
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