von Willerbrand diseases

posted on 25 Apr 2013 23:12 by ononmedicine in diesease
von Willerbrand diesease
เป็นโรคเลือดออกทางพันธุกรรม
โรคนี้มีแบบแผนการถ่ายทอดแบบ Autosomal dominant
ผู้ป่วยส่วนใหญ่ไม่มีความรุนแรงของโรคไม่มาก ทำให้ผู้ป่วยไม่ได้มาพบแพทย์
เป็นโรคอันดับสองรองจาก ฮีโมฟิเลีย เอ
เกี่ยวข้องกับโปรตีนที่ทำงานสัมพันธ์ใกล้ชิดกับ factor VIII
โดยเซลล์ที่สามารถสร้าง von willerbrand factor ได้แก่ endothelium และ megakaryocyte เท่านั้น
 
von Willerbrand เป็นโปรตีนที่มีความสำคัญในการห้ามเลือดสองประการ
1.ช่วยการเกาะติดของเกล็ดเลือดต่อเนื้อเยื่อเกี่ยวพันในบริเวณที่มีการฉีกขาดของหลอดเลือด (platelet adhesion)
โดยทำหน้าที่เหมือนสะพานเชื่อมระหว่างเกล็ดเลือด อาศัย A1 จับกับ glycoprotein Ib/IX/V complex บนผิวของ
เกล็ดเลือด กับเนื้อเยื่อเกี่ยวพัน
2.von willerbrand factor  จะจับกับ factor VIII ป้องกันไม่ให้ Factor VIII ถูกทำลายเร็วเกินไป ความผิดปกติจึงทำให้เลือดออกทั้งปฐมภูมิและทุติยภูมิ
 
von willerbrand สามารถแบ่งได้ 3 ชนิด
 
Type 1 ::: Quantiative defects
เป็นชนิดที่พบมากที่สุด ร้อยละ 60-80 มีการถ่ายทอดแบบ autosomal dominant
มักเป็นความผิดปกติบริเวณ promotor ส่วนมากเป็นแบบ gene deletion
ทำให้สร้างในปริมาณที่น้อยลง
 
Type 2 ::: Qualitative defects
พบประมาณร้อยละ 10-30 ของผู้ป่วยทั้งหมด
มีการถ่ายทอดพันธุกรรมเช่นเดียวกับ type 1 พบความผิดปกติที่นิวคลีโอไทด์บางตำแหน่ง
ทำให้ร่างกายสร้างคุณภาพผิดไปจากเดิม
 
Type 2A :::
เป็นความผิดปกติของ A2 domain ซึ่งเป็นบริเวณที่ถูก proteolysis ทำให้เกิดการย่อยสลาย
von willerbrand factor  มี 2 ประเภทที่แตกต่างกัน ทำให้ขาด vWF multimer เหมือนกันได้แก่
1.การเปลี่ยนแปลงของกรดอะมิโน บริเวณ A2 domain ทำให้ย่อยสลายvon willerbrand ง่ายขึ้น
ตรวจพบ vWF multimer ในเกล็ดเลือดแต่ไม่พบในพลาสมา
2.เกิดความผิดปกติในขบวนการ polymerization มีผลทำให้ vWF ไม่สามารถจับตัวเป็น multimer ได้
ทำให้ไม่มี vWF multimer และเกิดภาวะเลือดออกผิดปกติในที่สุด ตรวจไม่พบทั้งในพลาสมาและเกล็ดเลือด
 
Type 2B
เป็นความผิดปกติบริเวณ binding site ของเกล็ดเลือดทำให้ vWF จับกับเกล็ดเลือดได้มากขึ้น 
ทำให้จำนวนเกล็ดเลือดลดต่ำลง 
vWF ในพลาสมาลดลง แต่ในเกล็ดเลือดยังคงเป็นปกติ
 
Type 2M 
พบได้น้อยมาก ผิดปกติบริเวณ binding site ของเกล็ดเลือดทำให้ vWF ไม่สามารถจับกับเกล็ดเลือดได้
ทำให้เลือดออกผิดปกติอย่างรุนแรง
 
Type 2N
ความผิดปกติของกรดอะมิโนใน D domain Factor VIII ไม่มี vWF จับอยู่
Factor VIII ในคนไข้กลุ่มนี้จะต่ำมาก
 
Type 3
พบเพียงร้อยละ 1-5
มีอาการเลือดออกผิดปกติรุนแรง จะผิดปกติทั้งระดับและการทำหน้าที่ของ vWF รวมทั้ง multimer และ Factor VIII จะรุนแรงมาก ทำให้เลือดออกผิดปกติคล้าย  hemophilia A
 
[อาการ]
โดยมากแสดงออกจากความผิดปกติปฐมภูมิ 
1.จ้ำเขียวบริเวณที่กระแทกเบาๆ
2.เลือดออกตามไรฟัน
3.เลือดประจำเดือนออกมามากและนานกว่าปกติ
4.เลือดออกไม่หยุดหลังจากการถอนฟันหนือผ่าตัด
 
การตรวจแยกโรค
http://www.tsh.or.th/file_upload/files/vol16-3%2007%20von%20willebrand%20disease.pdf

edit @ 29 Apr 2013 11:59:54 by Ying_orn_lunlar

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