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PAROKSİSMAL SOĞUK HEMOGLOBİNÜRİSİ


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Medicine and Hematology Glossary

PAROKSİSMAL SOĞUK HEMOGLOBİNÜRİSİ

(Medicine and Hematology Glossary) :
Otoimmün hemolitik anemi grubunda. IgG antikorlarla oluşur. Eritrositlerdeki P antijenine spesifisite gösterir. Donath-Landsteiner antikorları olarak isimlendirilir. İdyopatik oloabilir veya viral infeksiyon seyrinde rastlanır. İlk tanımlandığı yıllarda en sık nedeni konjenital veya tersiyer sifiliz iken günümüzde sifilizin kontrol altına alınmış olmasıyla görülmüyor. Bugün sıklıkla genç yaşlarda viral infeksiyon sonrası görülür. Soğukla karşılaşınca ciddi hemoliz olur. Antikorlar bifazik özellik gösterir. Yani periferik dolaşımda 20 derecenin altında eritrosite bağlanır, kansantral dolaşıma taşındığında ise kompleman aktivasyonu ve hemoliz yapar.Donath-Landsteiner antikorları agglütinin özelliği göstermezler. Genellikle kendini sınırlayıcı bir hastalıktır. Gereken durumlarda transfüzyon yapılır. Sifilizebağlı olanlarda sifilizin etkin tedavisiyle geriler.
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