Hilmi Erdem SÜMBÜL, Mahmut BÜYÜKŞİMŞEK, Merve SARAÇOĞLU SÜMBÜL, Birol GÜVENÇ

Serebral venöz sinüs trombozu ile başvuran paroksismal noktürnal hemoglobinüri vakası

Venöz tromboembolizm (VTE), paroksismal nokturnla hemoglobinuri’de (PNH) morbidite ve mortalitenin önde gelen nedenidir. PNH hastalarının %29 ila %44'ü, karaciğer, beyin, bağırsak veya böbrekleri etkileyen ve klinik olarak belirgin bir VTE deneyimi yaşamaktadır. Bugüne kadar, omurilik ve kemik iliği dışındaki tüm ana organlarda VTE vakaları bildirilmiştir. Kavernöz venöz sinüs trombozu (KVST) nadirdir, ancak daha önce PNH seyrinde geç ortaya çıktığı bildirilmiştir. Burada, hastanemize ciddi sol taraflı temporal başağrısı, çift görme ve başlangıçta PNH'nin ilk belirtisi olarak görülen halsizlik, kırgınlık şikâyeti ile başvuran 34 yaşında bir kadın hastada bir KVST vakasını sunduk. Vakamız PNH’ın klinik tabloları içinde nadir görülen bir KVST hastasıdır. Bu vaka, PNH'lı hastalarda hemolitik aneminin eşlik ettiği diğer iki KVST vakası gibi PNH ilişkili sık gözlenen trombofilik durumu olmayan hastalarda PNH için farkındalığı teşvik etmektedir.

Anahtar Kelimeler:

Paroksismal nokturnla hemoglobinuria, Kavernöz venöz sinüs trombozu, Eculizumab

Paroxysmal nocturnal hemoglobinuria case presenting as cerebral venous sinus thrombosis

Venous thromboembolism (VTE) is the leading cause of morbidity and mortality in paroxysmal nocturnal hemoglobinuria (PNH). Between 29% and 44% of PNH patients experience a clinically evident VTE that affects the liver, brain, gut or kidney. Cases of VTE have been reported in all major organs except the spinal cord and bone marrow. Cavernous venous sinus thrombosis (CVST) is rare, but it has been reported previously to occur late in the course of PNH. Here we describe a case of CVST in a 34-year-old female admitted to our hospital with severe left sided temporal headache, double vision and malaise as an initial manifestation of PNH. This is a rare case of CVST as a presenting manifestation of PNH. This case along with two other recent reports of CVST accompanied by haemolytic anaemia in patients with PNH encourages increased vigilance for PNH in patients without an associated thrombophilic condition.

Keywords:

Paroxysmal nocturnal hemoglobinuria, Cavernous venous sinus thrombosis, Eculizumab,

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