文档介绍：DISORDERS OF HEMOSTASIS
Hematology ZhongShan Hospital,FuDan University
Prof. JlAN-MIN XU
General Considerations
Disorders of hemostasis
defects in platelet number or function
problems in fibrin clot (coagulation)
Bleeding due to platelet disorders is typically mucosal or dermatologic
include epistaxis, gum bleeding, menorrhagia, gastrointestinal bleeding, purpura, and petechiae
General Considerations
Petechiae are seen almost exclusively in conditions of thrombocytopenia and not platelet dysfunction.
Bleeding due to coagulopathy may occur as deep muscle hematomas as well as skin bleeding.
Spontaneous hemarthroses are seen only in severe hemophilia.
General Considerations
Definition:
disorder of hemostasis
spontaneous bleeding
abnormal bleeding following trauma
Machanism :
vessel wall defect
thrombocytopenia, platelet disfunction
coagulation disorder
Normal hemostasis and coagulation
hemostasis:
1. Vessel wall:
(1)vessel spasm: reflex spasm
S-HT↑ slow down blood flow
(2)subendothelial connective tissue:
encouraging platelet adhesion, activation,
aggregation
(3)releasing TF, activating FⅫ:
triggering intrinsic and extrinsic pathway
(4)pression of haematoma
Normal hemostasis and coagulation
2. platelet:
(1)adhesion:
to subendothelial connective tissue
by GPⅠb、vWF
(2)aggregation :
formation of platelet thrombus
by GPⅡb/Ⅲa
(3)release:
phosphatide-Arachidonic Acid -TXA2
further platelet activation and adhesion
violent vessel spasm
Normal hemostasis and coagulation
coagulation:
(1) Rearrangement of platelet membrane
phosphatidate-PF3
(2) Tissue injury- TF release
(3) Subendothelial connective tissue-Ⅻ-Ⅻa
triggering intrinsic and extrinsic pathway-white thrombus-red thrombus
Normal hemostasis and coagulation
coagulation:
nature: a cascade of enzymes activation
not active before ponent:
12 coagulation factor:
11 in plasma (tissue factor)
protein ( exclude Ca++ )
code with Ⅰ、Ⅱ、Ⅲ、Ⅳ etc.
PK
HMWK
Normal hemostasis an