Non-sirotik portal hipertansiyonun nadir bir nedeni: Primer antifosfolipid sendromu
Küçük Resim Yok
Tarih
2011
Dergi Başlığı
Dergi ISSN
Cilt Başlığı
Yayıncı
Erişim Hakkı
info:eu-repo/semantics/openAccess
Özet
Antifosfolipid sendrom, serumda uzun süre antifosfolipid antikorların varlığı, venöz ya da arteriyel trombozlar, tekrarlayıcı gebelik kayıpları veya trombositopeniyle karakterize sistemik otoimmün bir hastalıktır. İki formu tanımlanmıştır: başka hiç bir hastalığın eşlik etmediği birincil antifosfolipid sendrom ve genellikle sistemik lupus eritematozus gibi başka bir hastalığa eşlik eden ikincil antifosfolipid sendrom. Klasik özellikleri livedo retikularis, splinter hemorajiler, yüzeysel tromboflebit ve bacak ülserleri gibi deri bulguları; serebral, retinal ven trombozu gibi venöz trombotik olaylar; geçici iskemik atak ya da iskemik infarktlar gibi arteriyel trombotik olaylar, tekrarlayıcı erken-term spontan düşükler ve trombositopenidir. Budd-Chiari sendromu, hepatik veno-okluzif hastalık, küçük hepatik venlerin oklüzyonu, nodüler rejeneratif hiperplazi ve portal hipertansiyon gibi çeşitli karaciğer hastalıkları da antifosfolipid sendromu birlikteliğiyle literatürde bildirilmiştir. Biz de burada literatürde oldukça nadir görülen bir birliktelik olan primer antifosfolipid sendromuyla ilişkili non-sirotik portal hipertansiyon olgusu sunuyoruz.
The antiphospholipid syndrome involves long-term persistence of serum antiphospholipid antibodies and hypercoagulability manifested by venous or arterial thrombosis, recurrent pregnancy loss or thrombocytopenia. Two forms have been described: the “primary syndrome” where there is no evidence of an underlying disease, and the “secondary syndrome”, mainly in the setting of systemic lupus eryhematosus. Classic features of antiphospholipid syndrome include cutaneous signs, such as livedo reticularis, splinter hemorrhages, superficial thrombophlebitis, and leg ulcers; venous thrombotic events, such as cerebral or retinal vein thrombosis; arterial thrombotic events, such as transient attacks or ischemic infarcts, recurrent spontaneous early-term abortions, and thrombocytopenia. Also various hepatic manifestations have been reported with antiphospholipid syndrome in literature, including Budd-Chiari syndrome, hepatic-veno-occlusive disease and occlusion of small hepatic veins, nodular regenerative hyperplasia and portal hypertension. Herein we present a case of non-cirrhotic portal hypertension associated with primary antiphospholipid syndrome which this coexisting is rarely seen in the literature.
The antiphospholipid syndrome involves long-term persistence of serum antiphospholipid antibodies and hypercoagulability manifested by venous or arterial thrombosis, recurrent pregnancy loss or thrombocytopenia. Two forms have been described: the “primary syndrome” where there is no evidence of an underlying disease, and the “secondary syndrome”, mainly in the setting of systemic lupus eryhematosus. Classic features of antiphospholipid syndrome include cutaneous signs, such as livedo reticularis, splinter hemorrhages, superficial thrombophlebitis, and leg ulcers; venous thrombotic events, such as cerebral or retinal vein thrombosis; arterial thrombotic events, such as transient attacks or ischemic infarcts, recurrent spontaneous early-term abortions, and thrombocytopenia. Also various hepatic manifestations have been reported with antiphospholipid syndrome in literature, including Budd-Chiari syndrome, hepatic-veno-occlusive disease and occlusion of small hepatic veins, nodular regenerative hyperplasia and portal hypertension. Herein we present a case of non-cirrhotic portal hypertension associated with primary antiphospholipid syndrome which this coexisting is rarely seen in the literature.
Açıklama
Anahtar Kelimeler
Genel ve Dahili Tıp
Kaynak
Adnan Menderes Üniversitesi Tıp Fakültesi Dergisi
WoS Q Değeri
Scopus Q Değeri
Cilt
12
Sayı
3