Huy t h c
98
Hô I CH NG BERNARD–SOULIER
Hộ i chứng Bernard–Soulier là gì?
Là m t r i lo n ch y máu di truy n l n trên
NST thư ng.
Nguyên nhân
Là m t b nh di truy n mà h u qu gây thi u
h t glycoprotein (Gp) Ib.
C n lâm sàng:
- th i gian ch y máu; bình
thư ng ho c gi m s lư ng ti u c u.
•
B o t n:Gi i thích, d n dò BN.
•
Thu c:
○
Desmopressin có th làm gi m th i gian
ch y máu.
○
Y u t VIIa ho t hóa tái t h p (rFVIIa)
Hình thành cục máu đông
Bao g m 4 giai đo n. B t thư ng t
bư c 2-4 có th d n t i r i lo n ch y
máu.
1 Co m ch.
2 Ngưng t p ti u c u: B nh suy
như c ti u c u Glanzmann, b nh
von-Willebrand, h i ch ng
Bernard–Soulier.
3 T o c c máu đông: haemophilia.
4 Co c c máu: thi u h t antiplasmin.
HAEMOPHILIA
Hemophilia là gì?
Là m t b nh di truy n làm suy gi m
ch c
n
ăng đông máu.
Nguyên nhân
Là m t b nh di truy n. Có 2 lo i haemophilia:
•
Type A: thi u y u t VIII.
•
Type B: thi u y u t IX.
Xét nghi m
•
Th i gian prothrombin bình thư ng,
- th i gian thromboplastin t ng ph n ho t hóa.
Điều trị
•
L i s ng: gi i thích, d n dò BN. Tránh
các thu c aspirin, NSAIDs, heparin và
warfarin.
•
Thu c: thay th các y u t đông máu b
thi u h t b ng các ch ph m máu.
GLANZMANN’S THROMBASTHENIA
What is Glanzmann’s thrombasthenia?
This is a rare autosomal recessive or acquired
autoimmune condition in which platelets are
deficient of GpIIb/IIIa. GpIIb/IIIa binds fibrinogen.
Causes
Disease of hereditary or acquired
autoimmune cause.
Investigations
•
- Bleeding time.
Treatment
•
Conservative: patient education. Avoid
aspirin and nonsteroidal anti-inflammatory
drugs (NSAIDs).
•
Medical:
○
Desmopressin.
○
Recombinant activated factor VII.
VON WILLEBRAND DISEASE
What is von Willebrand disease?
This is the most common hereditary coagulation
disorder, which involves a defect in von
Willebrand factor (VWF). The function of von
Willebrand factor is to bind GpIb receptor on
platelets to subendothelial collagen.
Causes
Hereditary condition. There are many different
types of von Willebrand disease, but the most
common are type 1, type 2, type 3 and type
Normandy.
Investigations
•
- Activated partial thromboplastin time,
- Bleeding time, normal prothrombin time,
¯ VWF antigen, ¯ factor VIIIc.
Treatment
•
Conservative: patient education. Avoid aspirin
and NSAIDs.
•
Medical: desmopressin may be useful, but is
not helpful in type 3 von Willebrand disease.
VITAMIN K INSUFFICIENCY
What is vitamin K insuffiency?
This avitaminosis occurs when there is decreased
vitamin K
1
or vitamin K
2
or both. This results in:
•
¯ Synthesis of factors II, VII, IX and X.
•
¯ Synthesis of proteins C and S.
Causes
•
Drugs, e.g. warfarin.
•
Malnutrition.
•
Malabsorption.
•
Alcoholism.
•
Cystic fibrosis.
•
Chronic kidney injury.
•
Cholestatic disease.
Investigations
•
- Prothrombin time, normal or - partial
thromboplastin time.
Treatment
•
Conservative – patient education. Dietary
advice about food rich in vitamin K
•
Medical – treat cause. Vitamin K supplements.
MAP 6.3 R i lo n ch y máu
Map 6.3 R i lo n đông máu
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