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Haemostasis and Thrombosis - Coggle Diagram
Haemostasis and Thrombosis
Bleeding Disorders
Haemophilia A (FVIII deficiency)
Haemophilia B (FIX deficiency)
FXI deficiency
FXII deficiency
Von Willebrand Disease (VWD): Types 1, 2A, 2B, 2M, 2N, and 3
Factor XIII deficiency
Thrombocytopenia (various causes such as immune thrombocytopenic purpura (ITP), congenital, and platelet function defects)
Vitamin K deficiency
Glanzmann Thrombasthenia
Bernard-Soulier Syndrome
Wiskott-Aldrich Syndrome
Congenital and acquired factor deficiencies (e.g., FV, FX)
Thrombotic & Coagulation Disorders
Disseminated Intravascular Coagulation (DIC)
Protein S deficiency
Invasive Group A Streptococcal Sepsis with thrombosis and coagulopathy
Liver disease-related coagulation abnormalities
Haemostatsis
Physiological clot formation
Haemostasis screen
Prothrombin time (PT) (INR is internationally standardised PT)
Activated partial thromboplastin time (APTT)
Fibrinogen
Thrombin time
FBC platelet count
ISTH-BAT Bleeding assesment
Cutofss
6 - women
4 - men
3 - children
HX
PMHx
Known bleeding disoder
Hx of liver disease
Hx of CTD - connetive tissue disorder (SLE)
FHx
Bleeding disorders
Negative fhx foes not preclude
Meds Hx
Coagulation inhibition
Apixaban (Eliquis)
Dabigatran (Pradaxa)
Rivaroxaban (Xarelto)
Edoxaban (Lixiana)
Warfarin
Enoxaparin (Clexane)
Tinzaparin (Innohep)
Heparin
Collagen degradation
Corticosteroids
Platelet function inhibition
Aspirin
Clopidogrel (Plavix)
Nonsteroidal anti-inflammatory drugs
Fish oil
Selective serotonin reuptake inhibitors
Thpombocytopenia
Alcohol
Antibiotics (cephalosporins, linezolid, nitrofurantoin, penicillin, rifampin, sulfonamides, vancomycin)
Carbamazepine (Tegretol)
Quinine
Thiazide diuretics
Valproic acid
Evaluation of Prolonged PT/APTT screen
50:50 Mix with normal plasma
Corrected
Factor deficiency
Not corrected
Inhibitor
Cause of Prolonged APTT
P
lay
T
able
T
ennis inside
Intrinsic factors
Haemophilia A - Factor 8 deficiency
Haemophilia B - Factor 9 deficiency
Factor 11 deficiency
Factor 12 deficiency
Von Willebrand diseae
Causes of Bleed + Prolonged PT and APTT
1. Severe vit k deficiency
Deficiency of factors V, X, or II
Combined V + VIII deficiency
Inherited deficiency of enzymes involved in activation of vit k dependant clotting factors (1972)
Warfarin
Causes of Prolonged PT, APTT, and low Fibrinogen
Hypo/dysfibrinogenaemia
Liver disease
Hyperfibrinolysis
Following admit of streptokinase / tPA
Massive blood transfusion without adequate replacement of clotting factors including fibrinogen
DIC
Causes of Bleeding with Normal Screen
Factor 8 deficiency
Platelet function defect
Glanzmann thrombasthenia (GT)
Bernard-Soulier syndrome (BSS)
Mild VWD
Treatment with LMWH
Clincial Hx of Bleeding
Check FBC, Coag, vWF, Blood film
Thrombocytopenia
Abnormal morphology
Shistocytes: Microangiopathy
TTP, HUS, DIC
Blasts: Leukaemia
Micro thrombocytopenia with immunodeficiency: Wiskott-Aldrich syndrome
Macro thrombocytopenia : MYH6 disorders
Normal Morphology
ITP (Immune thrombocytopenia)
Congenital thrombocytopenia
Platelet function defect
Normal Platelet count
Normal Coag
Normal vWF
Check FXII (Factor 12)
Check platelet aggregometry
Check platelet immunophenotyping
Check platelet neuclotides
Abnormal Coag corrected with normal plasma
Or
Abnormal vWF screen
Bleedig disorder
vWD
Haemophilia
Haemophilia A
Factor 8 deficiency
Epidemiology
Incidence 1: 5,000 live
male
births
X linked - Xq28
Haemophilia B
Factor 9 deficiency
Epidemiology
1: 30,000 live
male
birhts
More common in ireland - 1: 12,5000
X linked - Xq27
Classification
Mild
5-14% factor activity
Cause of haemorrhage
Major trauma or surgery
Treatment
On demand post major trauma and peri-operative
Moderate
1-5% factor activity
Cause of haemorrhage
Minor trauma or surgery
Occasinonal spontaneous haemarthrosis
Treatment
On demand
Few require prophylaxis
Severe
<1% factor activity
Cause of haemorrhage
Lifelong spontaneous bleedings and haemarthroses starting in infancy
Treatment
Prophylaxis
Treatment
Replacement of missing clotting factor ( VIII or IX)
Von Willebrand Disease
Epidemiology
Most common inherited bleeding disorder
Up to 1% incidence
Can be AD or AR inheritance
Bleeding manifestations are mainly mucosal or post trauma / surgery
Classification
Type 3
Total
Quantative
deficiency
Type 2
Qualatative
defect
Type 1
Partial
quantative
deficiency
Breastfeeding and Vit K deficiency
Infants have low Vit k at birth - doesn't cross placenta
Low vit k in breast milk
Not in cows or formula
3 Sterility of foetal gut - low absorption
Coagulopathy / DIC
Diagnosis
Invasive Group A strep sepsis with thrombosis and coagulopathy
Possible aquired Protein S deficiency
Prolonged PT, APTT, low fibrinogen, thrombocytopenia, and positive D-dimer.
Treatment
Treat underlying causes
No need to correct abnormal coag tests unless bleeding
Anticoagulation
Triggeres
Sepsis
Malignancy
Trauma
Severe infx
Characterized by widespread activation of the coagulation cascade, leading to consumption of clotting factors and platelets.
