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Hemostasis

Isbandiyah,dr, SpPD
Definition of HEMOSTASIS
• The arrest of bleeding by repair of vessel wall
• Maintaining a balance
– Coagulation
– Fibrinolysis
• Hypocoagulation: excessive bleeding (inherited or
acquired)
• Hypercoagulation (thrombosis) inadequate activation
of the fibrinolytic system
HEMOSTATIC PROCESS

• Primary Hemostasis
– Blood vessel contraction
– Platelet Plug Formation

• Secondary Hemostasis
– Activation of Clotting Cascade
– Deposition & Stabilization of Fibrin

• Tertiary Hemostasis
– Dissolution of Fibrin Clot
– Dependent on Plasminogen Activation
Systems Involved in Hemostasis
• Vascular system
– Injured vessel initiates vasoconstriction
• Platelet System
– Injured vessel exposes collagen that initiates platelet
aggregation and help form plug
• Coagulation System
– protein factors of intrinsic and extrinsic pathways produce
a permanent fibrin plug
Hemostasis Lab Tests
•CBC-Plt
BV Injury •BT,(CT)
•PT
Tissue •PTT
Neural Platelet Factor
Adhesion
and
Activation

Blood Vessel Platelet Coagulation


Constriction Aggregation Cascade

Primary hemostatic
Reduced
plug Fibrin Plt Study
Blood flow formation Morphology
Function
Antibody
Stable Hemostatic Plug
Primary Hemostasis Secondary Hemostasis

Onset Immediate Delayed

Site Superficial Deep

Skin Petechiae, superficial Deep ecchymosis,


ecchymosis hematoma

Mucosal Common Rare

Others Rare Retroperitoneal


hematoma, hemarthrosis
Primary Hemostatic defect Secondary Hemostatic defect
Primary hemostasis
• vasoconstriction (vascular system)
• platelet exposure to subendothelial connective tissue
of blood vessels
• Platelet release of ADP, ATP, Thromboxane A2
(promotes vasoconstriction)
• Platelet aggregation, phospholipid provides site for
fibrin formation
Primary Hemostatic Disorders (PHD) and
Its Assessment
Platelet function (quantitative / qualitative)
Complete blood count (CBC)
Bleeding time
Platelet aggregation study
Blood vessel (vascular integrity)
Bleeding time
von Willebrand factor (vWF)
Bleeding time
vWF Antigen, vWF multimer, FVIII
Quantitative Platelet Problems
• Using too many/ increased destruction
– Immune destruction: ITP, SLE, Anti Platelets Ab
– Non Immune destruction: DIC, TTP
– Drug induced: heparin (HIT), H2 Blocker
– Sepsis
• Low produce
– Bone marrow problem: Aplastic Anemia, Amegakaryocytic
thrombocytopenia, infection, toxins, drugs, Infiltrative marrow disease
• Maldistribution
– Splenic sequestration
– Cirrhosis (Hypersplenism)
Qualitative Platelets Disorders
• Congenital / Genetic
– Von Willebrand’s disease
– Glanzmann’s Thrombasthenia
– Bernard-Soulier Syndrome, a Gp1b receptor deficiency
(can’t bind to each other)
• Acquired
– Aspirin, other anti platelets aggregation drugs !! *
– Renal Failure: Uremia Inhibits Gp1b

• If normal aPTT and normal BT, then normal platelets

* : Most of common cases in clinic


Principles treatment of PHD
• Thrombocytopenia:
– Treat underlying disease / causes of thrombocytopenia
– No bleeding, no TC Transfusion
– With bleeding: can be given TC transfusion until bleeding stop
• Thrombopathy:
– Congenital anomaly with bleeding: Tx. The disease according to its
guidelines (Platelet transfusion or Cryo or DDAVP)
– Acquired: Tx. Underlying disease, Stop anti platelet aggregation drugs
• Increase of vascular permeability:
– Tx. Underlying disease
– Give drugs that can reduce vascular permeability; e.g: Carbazochrome
(Crome®)
Idiopathic thrombocytopenic purpura (ITP)
• Annual incidence: 1/10,000 children

• Pathogenesis
– Accelerated destruction of Ab-sensitized platelets by phagocytic
cells (especially in the spleen)
– Gp IIb-IIIa: most common auto Ag

• Classification:
– Acute ITP : generally in children, preceding viral infection
– Chronic ITP: in adult (80%), thrombocytopenia > 6 months
• Management of ITP

– First-line therapy: steroids, IVIG


– Second-line therapy: cyclosporine, danazol, vinca alkaloids,
azathioprine, cyclophosphamide

– Splenectomy
• Not always curative, but usually promotes long-term and sustained
responses
• Complete remission rate: 72%
• Major risk: fatal sepsis due to encapsulated organisms
– Vaccination
– Penicillin prophylaxis

– Treatment decisions should be based on symptoms than on


platelet count !!

14
von Willebrand Disease
• The most common inherited bleeding disorder
• About 1% of the population
• Types I, II, and III.
• Symptom & sign: easy bruising, epistaxis, gum bleeding, menorrhagia,
post-partum hemorrhage, post-surgical or dental extraction bleeding
• Lab findings:
– BT prolonged, PT normal,
– APTT normal or prolonged

• Type I and III: reduced quantity of vWF


 TX: Cryo, DDAVP
A 27 y.o. male with type III vWD and a 2-
• Type II- defect in vWF molecule  TX: week duration of bleeding from the tongue.
Cryo Hemorrhage was controlled with
cryoprecipitate.
Secondary Hemostasis
Assessment of Secondary Hemostasis

Screening tests: Additional Tests


CT Fibrinogen
PT, INR Thrombin Time
aPTT Reptilase time
Mixing Study Coagulation factor
assays (F VIII)
D-dimer
Fibrin Degradation
Product
Euglobulin lysis time
Secondary Hemostatic Disorders
• Hemofilia A, B, C
• Vitamin K deficiency
– severe coagulation defect
– Required for synthesis of prothrombin and factors
VII, IX and X
• Parenchymal diseases of the liver
– Liver synthesizes several coagulation factors
• DIC (Mixed disorders)
Principles treatment of SHD
• Congenital anomaly: Substitute appropriate
coagulant-factor deficiency
• Acquired:
– Tx. Underlying disease / underlying causes
– Substitute Vit. K
– Substitute coagulant factors or Prothrombin-
complex concentrate
Hereditary deficiencies
• Hemophilia A: F.VIII • Hemophilia B: F.IX
deficiency deficiency
– Sex-linked recessive – Clinically indistinguishable from
– Hemarthroses, hematom are Hemophilia A
common – Sex-linked recessive
– Tx: F VIII : hRecombinant or cryo. – Need 50% preoperatively
– Need 100% levels preop, keep at – Prolonged PTT, normal PT
30% postop.
TX: Factor IX or FFP

Notes:
• FFP: Every ml contains 1 Unit of F.VIII
• Cryoprecipitate : Each bag contains 125 Units of F.VIII.
Vitamin K Deficiency
• Vitamin K is a fat-soluble vitamin that can be absorbed in the
presence of bile salts.
• Vitamin K is required for the production of coagulation factors
in the liver (factors II, VII, IX, X).

• Vitamin K deficiency  severe coagulation defect


Liver Disease
• Coagulation Disorders in Liver Disease:
– Decreased production of coagulation proteins (II,VII,IX,X),
and fibrinogen (factor I) and factor V
– Some degree of vitamin K deficiency, due to loss of storage
sites; e.g: liver cirrhosis
– Hypercoagulable state and predisposed to developing DIC
or systemic fibrinolysis.
DIC: Consumption Coagulopathy
• Severe DIC were accompanied with severe shock, blood stasis, caused by
systemic infection especially gram-negative organisms.

• Widspread / diffuse intravascular thrombosis will result in consumption of


coagulation especially factors I, II, V, VIII and thrombocytopenia with
secondary bleeding.

• Laboratory findings:
↓ platelets ,↓ fibrinogen, ↓ factors II, V, VII and ↑ FDP

• Treatment:
-Treatment underlying disease.
-Platelets and FFP transfusion.
-Heparin is restricted to widespread thrombosis e.g. purpura fulminans .
-Exchange transfusion with fresh blood in neonates.
Fibrinolysis
• Primary Fibrinolysis :
– excessive fibrinolysis caused by fibrinolytic enzyme (t-PA,
plasmin) in the circulation.
– e.g: thrombolytic treatment, APL (AML-M3), decreased
inhibitor (anti plasmin, PAI-1), Chronic liver disease

• Secondary Fibrinolysis: compensated response of DIC


Assessment of Fibrinolysis
• Circulating plasminogen activators or plasma: Euglobin lysis
time, t-PA activity, Plasmin-antiplasmin complex

• Proteolysis of coagulation factors by plasmin: PT, aPTT,


Fibrinogen, F.V, F.VIII

• Lysis of fibrin: FDP, D-Dimer,

• Fibrinolytic reactan consumption: Plasminogen, α2-


antiplasmin, PAI-1
Bleeding manifestation
• Ooze bleeding (primary fibrinolysis)
• Localized:
– Post operation bleeding
– Post invasive procedure bleeding
– Bleeding in place of IM injection
• Disseminated : Bleeding due to DIC (secondary
fibrinolysis)
Principles treatment of Fibrinolysis

• Tx. Underlying disease


• Stop thrombolytic agent
• Give anti fibrinolytic agent
• Correction of PHD or SHD
Tranexamic acid
- anti-fibrinolysis
- Adjunctive tx in areas with high
fibrinolysis (Oral cavity, GI tract,
GU tract)
- Contraindication : DIC,
Thrombosis, Renal bleeding
(obstructive uropathy)
- IV : 10 mg/kg/dose q 8 h
- Oral : 25 mg/kg/dose q 8 hr
- Oral wash in dental bleeding