Presentation and Management of Paroxysmal Nocturnal Hemoglobinuria: A Single-Center Experience

Autor: Orhan Ayyildiz, Abdullah Karakuş, Mehmet Sinan Dal, Mehmet Önder Ekmen
Jazyk: angličtina
Rok vydání: 2016
Předmět:
Zdroj: Hematology Reports; Volume 8; Issue 1; Pages: 6409
Hematology Reports, Vol 8, Iss 1 (2016)
Hematology Reports
ISSN: 2038-8330
DOI: 10.4081/hr.2016.6409
Popis: Paroxysmal nocturnal hemoglobinuria (PNH) is a rare acquired disorder characterized by intravascular hemolysis. Real-world experience of PNH management is largely unreported. A retrospective analysis was undertaken based on medical records from six patients with PNH [two with aplastic anemia (AA)] treated at our center, Dicle University, Turkey. Diagnosis was based on granulocyte PNH clones, ranging from 93% to 66%. All patients had symptoms consistent with PNH. One patient was managed adequately with supportive measures only. Five were treated with the complement inhibitor eculizumab. Follow-up data (
Databáze: OpenAIRE