Bleeding Disorder Flashcards
Haemorrhagic Diathesis
Any quantitative or qualitative abnormality due to inhibition of function of Platelets, vWF, coagulation factors.
Best questions to ask in terms of History of Bleeding
Bruising Epistaxis Post-surgical bleeding (dental surgery, circumcision, tonsillectomy, appendicectomy, adenoidectomy). Menorrhagia Post-partum haemorrhage Post-trauma Family history of bleeding disorder
How to determine the severity of bleeding
What makes you bleed. Is the severity of the bleeding out of context with the severity of the insult.
Platelet type bleeding disorder
Thrombocytopenia and Mucosal bleeding
- Epistaxis
- Purpura
- Menorrhagia
- GI
Coagulation bleeding disorder
Articular
Muscle Haematoma
CNS bleeding
Congenital or Acquired
Previous Episodes ?
Age at first event
Previous surgical challenges
Associated History
Age of presentation of haemophilia
Between 6 months and 2 years
Hereditary Disorder
Family members with a similar history and the sex of the people afflicted with haemophilia.
Haemophilia A (more common) and B
Both X-lined with identical phenotypes.
The severity of bleeding depends on the residual coagulation factor activity
<1% severe
1-5% moderate
5-30% mild
Prevalence of Haemophilia A
1:10,000
Prevalence of Haemophilia B
1:60,000
Clinical features of haemophilia
Haemarthrosis (especially in the hinge joint) Muscle Haematoma CNS bleeding Retroperitoneal bleeding Post surgical bleeding Muscle wasting in the calves and thighs
Knee damage in haemophilia
Acute inflammatory response, the macrophages produce pro-inflammatory cytokines which results in synovitis
End stage haemophilic arthropathy
Clinical Complications of Haemophilia
Synovitis in the knee
Chronic Haemophilic Arthropathy
Neurovascular Compression (compartment syndrome)
Stroke
Diagnosis of Haemophilia
Clinical Presentation - child fails to walk on limbs due to pain in joints
Prolonged APTT
Normal prothrombin time
Normal BT
Reduced FVIII or FIX
Genetic analysis
Haemophilia Treatment
Coagulation factor replacement FVIII/IX which are now mostly recombinant products.
Desmopressin (releases stored vWF into the circulation, especially useful in procedures)
Tranexamic Acid
Emphasis on using concentrates to reduce chance of bleeding
Synovectomy
Development in Inhibitors
AntiFVIII antibody development (30% of boys with severe haemophilia A)
von Willebrand Disease
Common (1in200)
Autosomal inheritance
Platelet type bleeding
Quantative (type 1) and qualitative (type 2 mutation in binding sites resulting in futile vWF) abnormalities of vWF
Type 3 vWD
Severe complete deficiency
Treatment of vWD
vWF concentrate or DDAVP
Tranexamic Acid
Topical applications
OCP
Cause of thrombocytopenia
Decreased production of platelets (marrow failure, aplasia, infiltration)
Increased consumption (immune ITP, non-immune DIC, hypersplenism)
Idiopathic thrombocytopenic purpura associated with
Infection esp EBV,HIV
Collagenosis
Lymphoma
Drug induced
Blood count Idiopathic thrombocytopenic purpura
Thrombocytopenia is the only feature in the blood
Increased platelet consumption despite normal bone marrow
Treatment of Idiopathic thrombocytopenic purpura
Steroids, IV IgG, splectomy, thrombopoietin analogues (eltrombopag, romiplostim stimulate platelet pre-cursor cells)
Liver Failure
Haemorrhagic and pro-thrombotic disorder. Reduced procoagulatns and anticoagulants in the liver results in either disorder. Renal failure, infection etc trigger
Treatment of Liver Failure
Replacement FFP, vitamin K, Factor II, VII, IX, X
Haemorrhagic Disease of the newborn
Immature coagulation systems. Vitamin K deficient diet. Resulting in fatal and incapacitating haemorrhage. Completely preventable by administration of vitamin K at birth.
