Frequency of and reasons for paroxysmal nocturnal haemoglobinuria screening in patients with unexplained anaemia
| Autor: | Heather A. Leitch, James T. England, Bakul I. Dalal |
|---|---|
| Rok vydání: | 2017 |
| Předmět: |
Adult
Male 0301 basic medicine medicine.medical_specialty Anemia Hemoglobinuria Paroxysmal Gastroenterology Pathology and Forensic Medicine 03 medical and health sciences hemic and lymphatic diseases Internal medicine Prevalence medicine Humans Practice Patterns Physicians' Aged Retrospective Studies Hematologic Tests biology business.industry Haptoglobin General Medicine Iron deficiency Middle Aged Eculizumab medicine.disease Pancytopenia Hemolysis 030104 developmental biology Immunology Paroxysmal nocturnal hemoglobinuria biology.protein Female business medicine.drug Anemia of chronic disease |
| Zdroj: | Journal of Clinical Pathology. 71:364-367 |
| ISSN: | 1472-4146 0021-9746 |
| DOI: | 10.1136/jclinpath-2017-204724 |
| Popis: | Referral to hematology for anemia is common. In paroxysmal nocturnal hemoglobinuria (PNH), cells deficient in the glycosylphosphatidyl inositol (GPI) anchor are lysed by complement. Eculizumab improves overall survival and quality of life while reducing hemolysis, transfusion requirements, and thrombosis. We evaluated the frequency of screening for PNH in patients with unexplained anemia. Key clinical features, laboratory data, and investigations were recorded for patients referred for anemia since 2010, without a specific cause found. PNH testing was done by flow cytometry. 540 patients had: anemia not yet diagnosed (NYD, n=318 (including unexplained iron deficiency, n=92; DAT-negative hemolysis, n=9)); anemia of chronic disease, n=173; and pancytopenia NYD, n=49. 82.4% had LDH testing done; 85.0% total bilirubin; 78.7% reticulocyte counts; and 40.6% haptoglobin level; 131 (24.2%) had possible hemolysis. PNH testing was done in 56 (10.4%). Those screened for PNH were more likely to have: younger age (P=0.04); a history of thrombosis (P |
| Databáze: | OpenAIRE |
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