Serebral venöz sinüs trombozu ile başvuran paroksismal noktürnal hemoglobinüri vakası

Yıl 2018, Cilt: 2 Sayı: 2, 190 - 192, 01.05.2018

Hilmi Erdem Sümbül , Mahmut Büyükşimşek , Merve Saraçoğlu Sümbül Birol Güvenç

https://doi.org/10.28982/josam.411600

Öz

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

Öz

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.

Anahtar Kelimeler

Paroxysmal nocturnal hemoglobinuria, Cavernous venous sinus thrombosis, Eculizumab

Kaynakça

• 1. Hillmen P, Lewis SM, Bessler M, Luzzatto L, Dacie JV. Natural history of paroxysmal nocturnal hemoglobinuria. N Engl J Med. 1995;333:1253-8.
• 2. Hillmen P, Muus P, Duhrsen U, Risitano AM, Schubert J, Luzzatto L, et al. Effect of the complement inhibitor eculizumab on thromboembolism in patients with paroxysmal nocturnal hemoglobinuria. Blood. 2007;110:4123-8.
• 3. Misra UK, Kalita J, Bansal V, Nair PP. Paroxysmal nocturnal haemoglobinuria presenting as cerebral venous sinus thrombosis. Transfus Med. 2008;18:308-11.
• 4. Sharma A, Itha S, Baijal SS, Gupta R, Sonkar A, Aggarwal R. Superior sagittal sinus thrombosis and Budd-Chiari syndrome due to paroxysmal nocturnal hemoglobinuria managed with transjugular intrahepatic portosystemic shunt: a case report. Trop Gastroenterol. 2005;26:146-8.
• 5. Zimmerman D. Bell WR. Venous thrombosis and splenic rupture in paroxysmal nocturnal hemoglobinuria. Am J Med. 1980;68:275-9.
• 6. Brodsky RA. How I treat paroxysmal nocturnal hemoglobinuria. Blood. 2009;13:6522-7.
• 7. Socie G, Mary JY, de Gramont A, Rio B, Leporrier M, Rose C, et al. Paroxysmal nocturnal haemoglobinuria: long-term follow-up and prognostic factors. French Society of Haematology. Lancet. 1196;348:573-7.
• 8. Lee JW, Jang JH, Kim JS, Yoon SS, Lee JH, Kim YK, et al. Clinical signs and symptoms associated with increased risk for thrombosis in patients with paroxysmal nocturnal hemoglobinuria from a Korean Registry. Int J Hematol. 2013;97:749-57.
• 9. Hill A, Kelly RJ, Hillmen P. Thrombosis in paroxysmal nocturnal hemoglobinuria. Blood. 2013;121:4985-96.
• 10. Audebert HJ, Planck J, Eisenburg M, Schrezenmeier H, Haberl R. Cerebral ischemic infarction in paroxysmal nocturnal hemoglobinuria report of 2 cases and updated review of 7 previously published patients. J Neurol. 2005;252:1379-86.
• 11. Moyo VM, Mukhina GL, Garrett ES, Brodsky RA. Natural history of paroxysmal nocturnal haemoglobinuria using modern diagnostic assays. Br J Haematol. 2004;126.133-8.
• 12. Haspel RL, Hillmen P. Which patients with paroxysmal nocturnal hemoglobinuria (PNH) should be treated with eculizumab? ASH evidence-based review 2008. Hematology Am Soc Hematol Educ Program. 2008;35.
• 13. Lazo-Langner A, Kovacs MJ, Hedley B, Al-Ani F, Keeney M, Louzada ML, Chin-Yee I. Screening of patients with idiopathic venous thromboembolism for paroxysmal nocturnal hemoglobinuria clones. Thromb Res. 2015 Jun;135(6):1107-9.
• 14. Hillmen P, Muus P, Roth A, Elebute MO, Risitano AM, Schrezenmeier H, et al. Long-term safety and efficacy of sustained eculizumab treatment in patients with paroxysmal nocturnal haemoglobinuria. Br J Haematol. 2013;162:62-73