Primary antiphospholipid syndrome patient associated with refractory thrombocytopenia and Factor V Leiden mutation treated with eltrombopag

Yıl 2017, Cilt: 10 Sayı: 1, 67 - 72, 01.04.2017

Sinem Namdaroğlu Şerife Solmaz Medeni Tuğba Çetintepe Ozan Barış Namdaroğlu Oktay Bilgir

Öz

Antiphospholipid antibody syndrome (APS) is a systemic autoimmune disorder characterized by recurrent arterial or venous thrombosis, fetal loss, thrombocytopenia, neurological symptoms and presence of serum antiphospholipid antibodies (AFA). In this report,we present a young male patient with thrombocytopenia who was refractory to steroids, intravenous immunoglobulin and splenectomy but had complete response to eltrombopag treatment, FV Leiden heterozygous mutant positive, also had a history of sinüs thrombosis taking oral anticoagulant treatment has been diagnosed primary APS. Eltrombopag treatment primary antiphospholipid syndrome patient associated with refractory thrombocytopenia.

Anahtar Kelimeler

Eltrombopag, Primary antiphospholipid syndrome, Thrombocytopenia, Factor V Leiden

Refrakter trombositopeni ile seyreden Faktör V Leiden mutasyonu olan primer antifosfolipid sendromlu olguda eltrombopag deneyimi

Öz

Antifosfolipid antikor sendromu (AFS), tekrarlayan arteriyel veya venöz trombozlar,fetal kayıplar,trombositopeni,nörolojik semptomlar ve serumda antifosfolipid antikor (AFA) varlığı ile karakterize sistemik otoimmün bir bozukluktur. Bu yazıda steroid,intravenöz immunglobulin ve splenektomi tedavilerine refrakter,eltrombobag tedavisine tam yanıtlı trombositopenisi olan,FV Leiden heterozigot mutant saptanan ve geçirilmiş sinüs ven trombozu olması nedeniyle ömür boyu oral antikoagulan tedavi alması gerekli primer AFS tanısı koyduğumuz genç bir erkek olgu sunulacaktır.

Anahtar Kelimeler

Eltrombopag, Antifosfolipid antikor sendromu, Trombositopeni, Faktör V Leiden