The Use of Plasma-Derived Factor VIII in Two Patients Diagnosed with TTP
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Tarih
2022
Yazarlar
Dergi Başlığı
Dergi ISSN
Cilt Başlığı
Yayıncı
Galenos Publ House
Erişim Hakkı
info:eu-repo/semantics/openAccess
Özet
Thrombotic thrombocytopenic purpura (TTP) develops due to increased von Willebrand factor multimers as a result of a deficiency of the a disintegrin and metalloproteinase with thrombospondin type 1 motif, member 13 enzyme. It has two forms; acquired or congenital (hereditary and familial). The initial clinical manifestations of this disease have been defined as a pentad consisting of thrombocytopenia, microangiopathic hemolytic anemia, neurological findings, acute renal failure, and fever. The basis of TTP therapy consists of fresh frozen plasma (FFP) and therapeutic plasma exchange (TPE). Our cases were two patients diagnosed with congenital TTP. Plasma-derived factor 8, which is a Factor VIII concentrate, was administered to these patients at a dose of 30 U/kg/week due to the allergic reactions the patients developed during their FFP and TPE treatments, prevention of exposure to the viral agent and ineffective treatment. After this treatment, laboratory parameters improved in case 1 and clinical improvement was achieved. In case 2, however, the desired level of laboratory parameters could not be reached and no clinical improvement was achieved.
Açıklama
Anahtar Kelimeler
TTP, plasma -derived factor VIII, ADAMTS-13
Kaynak
Cyprus Journal of Medical Sciences
WoS Q Değeri
N/A
Scopus Q Değeri
Cilt
7
Sayı
6
