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

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

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)] t...

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Veröffentlicht in:Hematology reports 2016-03, Vol.8 (1), p.6409-6409
Hauptverfasser: Dal, Mehmet Sinan, Karakuş, Abdullah, Ekmen, Mehmet Önder, Ayyildiz, Orhan
Format: Artikel
Sprache:eng
Schlagworte:
Aplastic anemia
Complement inhibitors
Dysphagia
eculizumab
Hemoglobin
Hemolysis
L-Lactate dehydrogenase
Lactic acid
Leukocytes (granulocytic)
Medical records
Paroxysmal nocturnal hemoglobinuria
Patients
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Zusammenfassung: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 (
ISSN:2038-8322
2038-8330
2038-8330
DOI:10.4081/hr.2016.6409