International Journal of Hematology and Oncology
2023, Vol 33, Num 4 Page(s): 128-132
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INHIBITORS IN HEMOPHILIA A AND B IN SOUTHEAST OF TURKEY
Abdullah ALTINTAŞ1, Orhan AYYILDIZ1, Timuçin ÇİL2, Murat SÖKER3, Ekrem MÜFTÜOĞLU1
1Dicle Üniversitesi, İç Hastalıkları, Hematoloji Bilim Dalı, DİYARBAKIR
2Dicle Üniversitesi, İç Hastalıkları Onkoloji Bilim Dalı, DİYARBAKIR
3Dicle Üniversitesi, Çocuk Hastalıkları Ana Bilim Dalı, DİYARBAKIR
Keywords: Hemophilia, Inhibitor, Bethesda test
Hemophilia A and B are inherited, X chromosome linked coagulation disorders that characterized by clinical signs and symptoms seen in males. The most important treatment related complication was viral infection in the past years, but this problem is overcoming by developing of modern factors concentrate which are available in the market, nowadays. However, developing inhibitor against concentrated factors that used is the most important problem, as in western countries. In this study, we aimed to evaluate the inhibitor developing rate in 73 hemophiliac pediatric and adult patients who have treated and followed up in Dicle University hematology polyclinics in Southeastern Anatolia. 62 (85%) of cases were hemophilia A and 11 (15%) were hemophilia B. Of hemophilia A patients 41 (66.2%) had severe and 17 (27.4%) had moderate disease. The same rates were 7 (63.6%) and 3 (27.3%) in hemophilia B patients, respectively. Only one patient (2.4%) with severe hemophilia A had high titrate of inhibitor positivity (17 BU/ml). Inhibitor was present in any of hemophilia B patients. This rate was lower than previously reported data from community of Turkey. We proposed that the most important cause of this low frequency of inhibitor is using fresh frosen plasma in treatment of those patients with bleeding.
Abdullah ALTINTAŞ1, Orhan AYYILDIZ1, Timuçin ÇİL2, Murat SÖKER3, Ekrem MÜFTÜOĞLU1
1Dicle Üniversitesi, İç Hastalıkları, Hematoloji Bilim Dalı, DİYARBAKIR
2Dicle Üniversitesi, İç Hastalıkları Onkoloji Bilim Dalı, DİYARBAKIR
3Dicle Üniversitesi, Çocuk Hastalıkları Ana Bilim Dalı, DİYARBAKIR
Keywords: Hemophilia, Inhibitor, Bethesda test
Hemophilia A and B are inherited, X chromosome linked coagulation disorders that characterized by clinical signs and symptoms seen in males. The most important treatment related complication was viral infection in the past years, but this problem is overcoming by developing of modern factors concentrate which are available in the market, nowadays. However, developing inhibitor against concentrated factors that used is the most important problem, as in western countries. In this study, we aimed to evaluate the inhibitor developing rate in 73 hemophiliac pediatric and adult patients who have treated and followed up in Dicle University hematology polyclinics in Southeastern Anatolia. 62 (85%) of cases were hemophilia A and 11 (15%) were hemophilia B. Of hemophilia A patients 41 (66.2%) had severe and 17 (27.4%) had moderate disease. The same rates were 7 (63.6%) and 3 (27.3%) in hemophilia B patients, respectively. Only one patient (2.4%) with severe hemophilia A had high titrate of inhibitor positivity (17 BU/ml). Inhibitor was present in any of hemophilia B patients. This rate was lower than previously reported data from community of Turkey. We proposed that the most important cause of this low frequency of inhibitor is using fresh frosen plasma in treatment of those patients with bleeding.
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