Refractory autoimmune cytopenias in pediatric Evans syndrome with underlying systemic immune dysregulation
| Autor: | Jenny M. Despotovic, Susan E Kirk, Taylor O Kim, Michele P. Lambert, Jonathan M. Flanagan, Amanda B. Grimes, Rachael F. Grace |
|---|---|
| Rok vydání: | 2021 |
| Předmět: |
Adult
Male Evans syndrome Adolescent medicine.medical_treatment medicine.disease_cause Autoimmune Diseases Targeted therapy 03 medical and health sciences 0302 clinical medicine Refractory medicine Rheumatologic disease Humans Child Retrospective Studies business.industry Common variable immunodeficiency Immune regulation Infant Hematology General Medicine Immune dysregulation medicine.disease Thrombocytopenia Child Preschool 030220 oncology & carcinogenesis Autoimmune lymphoproliferative syndrome Immunology Female Anemia Hemolytic Autoimmune business 030215 immunology |
| Zdroj: | European Journal of Haematology. 106:783-787 |
| ISSN: | 1600-0609 0902-4441 |
| DOI: | 10.1111/ejh.13600 |
| Popis: | Evans syndrome is a rare but challenging disorder in children; and despite rapidly growing evidence for targetable systemic immune dysregulation driving these "idiopathic" autoimmune cytopenias, precision diagnosis and management remains sub-optimal among these patients. We analyzed retrospective clinical data for 60 pediatric ES patients followed at 3 large tertiary referral centers in the United States over a recent 6-year period and found that definable underlying systemic immune dysregulation was identified in only 42% of these patients throughout the course of clinical care. Median time from ES diagnosis to identification of the underlying systemic immune dysregulation disorder was 1.3 years ( |
| Databáze: | OpenAIRE |
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