Presentation and Management of Paroxysmal Nocturnal Hemoglobinuria: A Single-Center Experience
Autor: | Orhan Ayyildiz, Abdullah Karakuş, Mehmet Sinan Dal, Mehmet Önder Ekmen |
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Jazyk: | angličtina |
Rok vydání: | 2016 |
Předmět: |
Abdominal pain
medicine.medical_specialty aplastic anemia paroxysmal nocturnal hemoglobinuria Single Center Hemolysis Article 03 medical and health sciences Complement inhibitor 0302 clinical medicine Internal medicine hemic and lymphatic diseases medicine Aplastic anemia Adverse effect hemolysis eculizumab business.industry lcsh:RC633-647.5 Hematology lcsh:Diseases of the blood and blood-forming organs Eculizumab medicine.disease Dysphagia Surgery 030220 oncology & carcinogenesis Paroxysmal nocturnal hemoglobinuria medicine.symptom business 030215 immunology medicine.drug |
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 |
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