Bleeding

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Bleeding Disorders

Dr Fatima Abuzaid
Content
• Background and important definitions.
• Rapid review of Hemostasis.
• Recognition of Bleeding presentation.
• Explain systematic approach to bleeding child.
• Categorize Hematological and non
hematological cause of bleeding.
• Overview of treatment.
Background
• Bleeding: Extravasations of blood.
Important definitions
• Bleeding time: Time needed to stop bleed
after an injury(usually 3-5 minutes).
• Clotting Time: Time required to form clot
(normal 5-15minutes)
• Pro-Coagulant: Inactive form of coagulant
proteins.
• Coagulation cascade: chain of chemical
reaction to activate pro-coagulant and end
result transform pro-thrombin to thrombin.
Hemostasis
Thrombosis Bleeding
Following an Injury
• Vasospasm.
• Platelet plug formation : Collagen exposure
Platelet Adhesion(VWB), Platelet activation
secret ADP, serotonin, Tromboxane A2 lead to
platelet aggregation.
• Coagulation
Coagulation cascade
Causes of Bleeding
• Vascular:
– Vasculitis; e.g HSP.
– Vascular malformation: Hereditary Telangectasia.
– Extravascular; Ehlers Danlos syndrome
• Platelet:
– Low Number
– Dysfunction
• Coagulation:
– Intrinsic
– Extrinsic
– Both
Platelet defect
• Low Number:
– Decrease production(Aplastic, Acute Leukemia)
– Increase destruction(ITP,SLE)
– Consumption(DIC,HUS)

• Abnormal function:
– Bernard Soulier syndrome
– Glanzman thrombosthenia
– Others; Uremia, Drugs..etc
Coagulation defect
• VWB deficiency.
• Hemophilia A.
• Hemophilia B.
• Factor VII.
• Factor XIII.
• Complex factor deficiency.
• Others( Liver disease, drugs, DIC..etc)
History
• Onset, Trauma, Amount, site, Course, Duration.
• Site; Epistaxis, Hematemesis, Melena, Bruises
• Red flags(weight loss, Bone pain, Fever and night
sweat).
• Lethargy, Pallor, Breathlessness, Abdominal pain,
Confusion.
• History of URTI.
• PMHx (vaccination, Circumcision, Drugs)
• Family history.
Examination
• Local examination.
• Vital signs(CRT,HR,RR, Bp).
• Lymph nodes, Spleen, Liver(stigmata of liver
disease).
• Anthropometric measurements and skin.
• Systemic examination(Musckuloskeletal).
Investigation
• CBC, DCT, Blood film, viral screen, blood group,
cross match, Crp and Septic screen.
• Bleeding time.
• PTT,PT, and INR.
• Liver function.
• D.dimer, Fibrinogen.
• Platelet function(aggregation).
• Factor levels.
• BMA & Biopsy.
Treatment
Treatment
• ABC.
• Compression(open wound).
• Local treatment with Tranixemic acid.
• Check vitals assess hemodynamic state.
• Consider blood transfusion.
• Tranixemic acid IV.
• If overdose or poison > Antidote.
• Replacement therapy(concentrate/CPP/FFP).
Treatment
• ITP: Steroid, IV IG
• Aplastic Anemia; PlT transfusion(def BMT)
• Thrombosthenia; Platelet transfusion.
• Leukemia: Chemotherapy, BMT.
• N.B; PLT transfusion is contraindicated in ITP
unless life threatening bleeding.
Case scenario
• 2 year old boy presented with Right Knee
swelling, restriction of movement no fever.

• Take focused history


Investigation
• CBC
• WBC =5000 HGB=10 Plt=210
• PT=13 PTT=68 INR=1.2

• Factor VIII <1%


Hemophilia
• X linked recessive disease
• A Factor VIII deficiency
• B Factor IX deficiency
• Heamarthrosis, Hematoma, circumcision and
post vaccination bleeding.
• Prolonged PTT
• Mild 5-30% Moderate 1-5% Sever <1%
• Treatment: Factor VIII or IX.
ITP
• Idiopathic, auto-immune in nature
• Usually proceeded by URTI
• Skin and mucus membrane bleedin,
sometimes Heamturia and CNS bleeding.
• Diagnosis of exclusion.
• Blood film shows large platelet.
• Treated with IVIG or Steroids
• Second line Eltrobopag.
Questions?

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