Von Willebrand hastalığında tiplere göre uygulanan tedavi etkinliğinin retrospektif olarak araştırılması
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Dosyalar
Tarih
2022
Yazarlar
Dergi Başlığı
Dergi ISSN
Cilt Başlığı
Yayıncı
Ege Üniversitesi, Tıp Fakültesi
Erişim Hakkı
info:eu-repo/semantics/openAccess
Özet
AMAÇ: Çalışmamızın amacı, von Willebrand hastalığında (vWH) tiplere göre uygulanan tedavinin etkinliğinin, demografik özelliklerin, ailede vWH tanısı ya da başka kanama diyatezi varlığının, hastanın ilk semptomunun ve hastada gelişen tüm semptomların, ilk semptom başlama yaşının, tanı yaşının, tanı anındaki vWF:Ag, vWF:Ricof, FVIII, PFA Kollajen-ADP, PFA Kollajen-Epinefrin, PZ, aPTZ, PLT değerlerinin, komplikasyonların, geçirilmiş operasyonların değerlendirilmesidir. YÖNTEM: Ege Üniversitesi Çocuk Hematoloji Bilim Dalında 1980-2021 yılları arasında vWH tanısı almış hastalardan tip 1’den 50 kişi, tip 2’den 25 kişi, tip 3’den 15 kişi taranarak değerlendirildi. Olguların yaşı, boyu, kilosu, vWH öyküsü, başka kanama diyatezi öyküsü, hastanın ilk semptomu, ilk semptom başlama yaşı, hastada gelişen tüm semptomlar (burun kanaması, cilt kanaması, ağız içi kanama, diş çekimi sonrası kanama, cerrahi sonrası kanama, menoraji, diğer), tanı yaşı, tanı anındaki vWF:Ag, vWF:Ricof, FVIII, PFA Kollajen-ADP, PFA Kollajen-Epinefrin, PZ, aPTZ, PLT değeri, komplikasyonlar ve hastanın kullanmış olduğu tedaviler retrospektif olarak tarandı. Toplanmış bu veriler SPSS 26 istatistik programına
girilerek değerlendirildi. Kategorik değişenler sayı ve yüzdelerle, sürekli değişenler ise
ortanca ve ortalamalarla özetlendi. Sürekli değişenler Mann-Whitney U testi, kategorik değişenler ise Ki-kare ve Fisherin kesin testi kullanılarak karşılaştırıldı. Yapılan bu istatistiklerde anlamlılık düzeyi p<0.05 olarak kabul edildi. BULGULAR: Ege Üniversitesi Çocuk Hematoloji Bilim Dalında 1980-2021 yılları arasında vWH tanısı almış 90 hasta değerlendirildi. Çalışmaya dahil edilen 90 hastanın 50’si tip 1 vWH (%55,6), 25’i tip 2 vWH (%27,8), 15’i tip 3 vWH (%16,7) idi. Hastaların 44’ü erkek (%48,9), 46’sı kadındı (%51,1). Hastaların yaş ortalaması 18,5±7,2 (3-37) idi. Tipler arasında ilk semptom başlama yaşı ve tanı yaşı açısından tip 3 ile diğer tipler arasında istatiksel anlamlı fark saptandı. İlk semptom açısından istatiksel anlamlı fark saptanmadı. Tipler arasında vWF:Ag, vWF:Ricof, FVIII düzeyi, PFA 100 Kollajen-ADP ve aPTZ değeri açısından tip 3 ile diğer tipler arasında istatiksel anlamlı fark saptandı. PFA 100 Kollajen-Epinefrin değeri açısından tip 1 ile tip 3 arasında ise istatiksel anlamlı fark saptandı. SONUÇ: Sonuç olarak tedavi yaklaşımı vWH’nın tipine ve şiddetine göre değişir. Mukozal kanamalarda transamin, minör cerrahi, diş çekimi öncesinde transamin ve desmopressin, kadınlarda menoraji gibi durumlarda antifibrinolitik tedavi ve östrojen tedavisi uygulanmaktadır. Major cerrahilerde ve diğer tedavilere yanıtsız major kanamalarda vWF tedavisi kullanılmaktadır. Tip 1 ve bazı tip 2A vakalarında desmopressin tedavisi kulanılmaktadır. Desmopressinin yeterli olmadığı hastalarda ve diğer tip 2 ve tip 3 vWH’da vWF/FVIII konsantreleri tedavide daha etkili olmaktadır.
AIM: The aim of our study was to determine the effectiveness of the treatment applied according to the types in von Willebrand’s disease (vWD), demographic characteristics, the diagnosis of vWD in the family or the presence of other bleeding diathesis, the first symptom of the patient and all the symptoms developing in the patient, the age of first symptom onset, the age of diagnosis, at the time of diagnosis vWF:Ag, vWF:Ricof, FVIII, PFA Collagen-ADP, PFA Collagen-Epinefrin, PT, aPTT, PLT values, complications, previous operations. METHODS: Patients who 50 cases from type 1, 25 cases from type 2, 15 cases from tip 3 diagnosed with vWD at Ege University Department of Pediatric Hematology between 1980 and 2021 were evaluated screening. Age, height, weight of the cases, history of vWD, history of other bleeding diathesis, first symptom of the patient, age of first symptom onset, all symptoms developed in the patients (nose bleeding, skin bleeding, oral bleeding, bleeding after tooth extraction, bleeding after surgery, menorrhagia, other), age at diagnosis, at the time of diagnosis vWF:Ag, vWF:Ricof, FVIII, PFA Collagen-ADP, PFA-Collagen-Epinefrin, PT, aPTT, PLT values, complications and treatments used by the patient scanned retrospectively. These collected data were evaluated by entering the SPSS 26 statistical program. Categorical variables were summarized as numbers and percentages, and continuous variables were summarized as medians and means. Continuous variables were compared using the Mann-Whitney U test, and categorical variables were compared using Chi-square and Fisher's exact tests. The level of significance in these statistics was accepted as p<0.05. RESULTS: Ninety patients diagnosed with vWD in Ege University Pediatric Hematology Department between 1980-2021 were included. Of the 90 patients included in the study, 50 had type 1 vWD (55.6%), 25 had type 2 vWD (27.8%) and 15 had type 3 vWD (16.7%). Of the patients, 44 (48.9%) were male and 46 (51.1%) were female. The mean age of the patients was 18.5±7.2 (3-37) years. There was a statistically significant difference between type 3 and other types in terms of the age of first symptom onset and age at diagnosis. There was no statistically significant difference in terms of the first symptom. A statistically significant difference was found between type 3 and other types in terms of vWF:Ag, vWF:Ricof, FVIII level, PFA 100 Collagen-ADP and aPTT value. A statistically significant difference was found between type 1 and type 3 in terms of PFA 100 Collagen-Epinephrine value. CONCLUSIONS: As a result, the treatment approach varies according to the type and severity of vWD. Transamin in mucosal hemorrhages, transamin and desmopressin in minor surgery, before tooth extraction, antifibrinolytic therapy and estrogen therapy in cases such as menorrhagia in women are used. vWF treatment is used in major surgeries and in major bleeding unresponsive to other treatments. Desmopressin therapy is used in type 1 and some type 2A cases. vWF/FVIII concentrates are used in the treatment of patients in whom desmopressin is not sufficient and other type 2 and type 3 vWD.
AIM: The aim of our study was to determine the effectiveness of the treatment applied according to the types in von Willebrand’s disease (vWD), demographic characteristics, the diagnosis of vWD in the family or the presence of other bleeding diathesis, the first symptom of the patient and all the symptoms developing in the patient, the age of first symptom onset, the age of diagnosis, at the time of diagnosis vWF:Ag, vWF:Ricof, FVIII, PFA Collagen-ADP, PFA Collagen-Epinefrin, PT, aPTT, PLT values, complications, previous operations. METHODS: Patients who 50 cases from type 1, 25 cases from type 2, 15 cases from tip 3 diagnosed with vWD at Ege University Department of Pediatric Hematology between 1980 and 2021 were evaluated screening. Age, height, weight of the cases, history of vWD, history of other bleeding diathesis, first symptom of the patient, age of first symptom onset, all symptoms developed in the patients (nose bleeding, skin bleeding, oral bleeding, bleeding after tooth extraction, bleeding after surgery, menorrhagia, other), age at diagnosis, at the time of diagnosis vWF:Ag, vWF:Ricof, FVIII, PFA Collagen-ADP, PFA-Collagen-Epinefrin, PT, aPTT, PLT values, complications and treatments used by the patient scanned retrospectively. These collected data were evaluated by entering the SPSS 26 statistical program. Categorical variables were summarized as numbers and percentages, and continuous variables were summarized as medians and means. Continuous variables were compared using the Mann-Whitney U test, and categorical variables were compared using Chi-square and Fisher's exact tests. The level of significance in these statistics was accepted as p<0.05. RESULTS: Ninety patients diagnosed with vWD in Ege University Pediatric Hematology Department between 1980-2021 were included. Of the 90 patients included in the study, 50 had type 1 vWD (55.6%), 25 had type 2 vWD (27.8%) and 15 had type 3 vWD (16.7%). Of the patients, 44 (48.9%) were male and 46 (51.1%) were female. The mean age of the patients was 18.5±7.2 (3-37) years. There was a statistically significant difference between type 3 and other types in terms of the age of first symptom onset and age at diagnosis. There was no statistically significant difference in terms of the first symptom. A statistically significant difference was found between type 3 and other types in terms of vWF:Ag, vWF:Ricof, FVIII level, PFA 100 Collagen-ADP and aPTT value. A statistically significant difference was found between type 1 and type 3 in terms of PFA 100 Collagen-Epinephrine value. CONCLUSIONS: As a result, the treatment approach varies according to the type and severity of vWD. Transamin in mucosal hemorrhages, transamin and desmopressin in minor surgery, before tooth extraction, antifibrinolytic therapy and estrogen therapy in cases such as menorrhagia in women are used. vWF treatment is used in major surgeries and in major bleeding unresponsive to other treatments. Desmopressin therapy is used in type 1 and some type 2A cases. vWF/FVIII concentrates are used in the treatment of patients in whom desmopressin is not sufficient and other type 2 and type 3 vWD.
Açıklama
Anahtar Kelimeler
Von Willebrand Hastalığı, Von Willebrand Faktörü, Tedavi, Von Willebrand’s Disease, Von Willebrand Factor, Treatment
