Common Bleeding
Disorders
Dr Chanda Kapoma
Fifth Years
2 Objectives
By the end of this lecture the student should be able to:
Describe the basics of haemostasis
Discuss the clinical manifestations of:
ITP
Haemophilia
Vitamin K Deficiency Bleeding
Mention the investigations and treatment of these disorders
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3 Presentation Outline
Definition of Bleeding Disorders
Stages of Coagulation
Intrinsic and Extrinsic pathways of Coagulation
Epidemiology of Bleeding Disorders
ITP
Haemophilia
Vitamin K Deficient Bleeding
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4 What is haemostasis?
Haemostasis is the prevention of blood loss
Haemostasis is achieved by several mechanisms:
Vascular constriction
Formation of a platelet plug
Formation of a blood clot
Growth of fibrous tissue
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5 Steps of Coagulation
Formation of the prothrombin activator
Conversion of prothrombin into thrombin
Conversion of fibrinogen into fibrin fibres
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6 Steps of Coagulation
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7 How is clotting initiated?
1. Extrinsic pathway
2. Intrinsic pathway
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8 Extrinsic pathway
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9
Intrinsic pathway
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10 Tissue Factor and Factor XII
In the absence of calcium clotting does not take place
regardless of the pathway
Tissue factor initiates the extrinsic pathway
Contact of Factor XII and platelets with collagen in the
vascular wall initiates the intrinsic pathway
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11 Epidemiology of Bleeding Disorders
The three most common hereditary bleeding disorders are Hemophilia A,
Hemophilia B and von Willebrand Disease
The prevalence of Hemophilia increases where there are higher leves of
consanguinity
Von Willibrand Disease is autosomal dominant with no predilection for sex
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12 Classification of Bleeding Disorders
Blood Vessel Platelet Abnormality Abnormal Coagulation
Abnormality
Scurvy Thrombocytopenia: Congenital:
• Malignancy • Haemophilia A
• Viral infections • Haemophilia B
• Marrow damage
due to drugs
• Aplastic Anaemia
• ITP
• DIC (Septicaemia)
Septic or Toxic Vasculitis: Abnormal Platelet Acquired:
• Meningococcemi Function: • Haemorrhagic Disease
a • Von Willebrand’s of the Newborn
• Yellow fever Disease
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Anaphylactoid purpura
(HSP)
13
What is ITP?
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Immune ThrombcytoPenia
It is the most common platelet disorder in children
It is characterized by isolated thrombocytopenia
The cause remains unknown but it can be triggered by a viral
infection or other immunologic or environmental event
It has an immune mediated mechanism
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15 Pathogenesis of ITP
Autoantibodies are directed against platelet membrane
antigens shortening their half life
The same antibodies may inhibit platelet production
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16 Clinical Features of ITP
Sudden appearance of petechial rash, bruising and/or
bleeding in an otherwise healthy child
Preceded by a viral illness within the past month
Patients appear well and systemic symptoms are
generally absent
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17 Clinical Features of ITP
No significant enlargement of lymph nodes, liver or
spleen
Intracranial haemorrhage is a rare complication seen in
less than 1%
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18 Hae
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19 Laboratory Findings
Investigation Results
Full Blood Count Thrombocytopenia
Peripheral Blood Smear Platelets are decreased in number but
of normal size
Bone marrow Normal
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20 Treatment
General Measures:
Activity restriction
Avoidance of anticoagulant and antiplatelet medications
Control of menses
Education
Specific therapy will depend on the severity of the
bleeding
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21 Treatment
Life threatening bleeding:
Platelet transfusions
Methylprednisolone
Intravenous Immunoglobulin
When a rapid rise in the platelet count is not required, an
oral glucocorticoid like Prednisolone can be used
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22 Oral glucocorticoids
Prednisolone 4 mg/kg per day orally for 7 days then
rapid tapering
Prednisolone 1-2 mg/kg per day orally for 7-21 days then
tapering
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23 Prognosis
Most children resolve within 3-6 months with or without
treatment
10-20 % develop chronic ITP
Chronic ITP is thrombocytopenia persisting beyond 12
months since presentation
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25 What is haemophilia?
It is an inherited bleeding disorder caused by deficiency
of factor VIII (Hemophilia A), factor IX (Haemophilia B) or
factor XI (Haemophilia C)
Haemophilia A is more common than Haemophilia B
Both Haemophilia A and B are inherited in an X-linked
recessive manner
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27 Pathophysiology
Factors VIII and IX together with phospholipid and
calcium form the factor X activating complex
In Haemophilia A or B, clot formation is delayed and it is
not robust
There is inadequate thrombin formation and hence a
tightly linked cross fibrin clot is not generated
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28 Clinical Manifestations
Symptoms may occur at birth or in the fetus
30% of male infants bleed with circumcision
Easy bruising, intramuscular hematomas and
hemarthroses occur when the child begins to cruise
Hemarthrosis is the hallmark of haemophilia
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29 Clinical Manifestations
Hemarthroses occur initially in the ankle joints then
become more frequent in the knees and elbows
Patients complain of a warm, tingling sensation before
swelling becomes apparent
A joint can develop increased susceptibility to bleeding
once joint damage and inflammation occur (target
joint)
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30 Clinic Manifestations
Patients may lose large volumes of blood into iliopsoas
muscle with only a vague referred pain in the groin
The diagnosis is made clinically by the inability to extend
the hip and the maintenance in internal rotation
Intracranial bleeding is rare but it is one of the most life
threatening events in hemophilia
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31 Laboratory Findings
Prolonged activated partial thromboplastin time (aPTT)
Normal platelet and prothrombin time
Measurement of factor activity level is reduced (< 40 %)
Normal plasma von Wilebrand antigen factor (VWF: Ag)
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32 Classification of Hemophilia
Severe hemophilia
< 1 % of activity of the clotting factor: spontaneous bleeding occurs
Moderate hemophilia
1-5% and require moderate trauma to induce bleeding
Mild haemophilia
> 5% and may go many years before the condition is diagnosed
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33 Treatment
For mild to moderate bleeding, factor levels must be
raised to hemostatic levels of 30-50%
For life threatening or major haemorrhage, the dose
should aim to reach levels of 100%
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34 Dose of Recombinant Factor
Factor VIII (IU) = % desired (rise in FVIII) X body weight (kg) X 0.5
Factor IX (IU)= % desired (rise in FIX) X body weight (kg) X 1.4
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35 Prophylaxis
It has become the standard in management of patients
with severe haemophilia
Prophylaxis prevents spontaneous bleeding and early
joint deformity
Desmopressin Acetate can be used in those with mild
hemophilia
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36 Haemorrhagic Disease of the Newborn
There is a transient decrease in factors II, VII, IX and X in
all newborns by 48-72 hours after birth
There is a gradual return to birth levels by 7-10 days
This is due to lack of free vitamin K from the mother and
absence of intestinal bacterial flora
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37 Classification
• 0-24 hrs
Early Onset • Associated with maternal drugs
• 2-7 days
Classic • In neonates who do not receive Vit K
• 1-6 months
Late Onset • Associated with liver disease
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38 Classic Disease
The bleeding is gastrointestinal, nasal, subgaleal,
intracranial or postcircumcision
Breastfed neonates are more at risk
The PT, blood coagulation time and aPTT are prolonged
Factors II, VII, IX and X are significantly decreased
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39 Differential Diagnosis
Disseminated Intravascular Coagulation
Swallowed blood syndrome
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40 Treatment
IM administration of Vitamin K, 1 mg prevents the
decrease in vitamin K dependant factors
It may be treated with a subcutaneous injection of
Vitamin K1, 1-5 mg
Serious bleeding in premature infants and those with liver
disease may require fresh frozen plasma
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41
References
Guyton Textbook of Medical Physiology
Diseases of Children, Stanfield Paget
Hemophilia, Bree Zimmerman, MD Paediatrics in Review Vol 34, No 7, July
2013
Nelson Textbook of Paediatrics, 18th Edition
UpToDate Clinical Manifestations and diagnosis of Haemophilia W Keith
Hoots, MD, Amy D Shapiro, MD
UpToDate Treatment of bleeding and perioperative management in
Haemophilia A and B
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