15.8.3 Bleeding Disorders In Chidren Flashcards
Define haemostasis
- cessation/stop bleeding
- Process of keeping blood within the vessels
- by repairing damaged vessels,
- without compromising the flow of the blood
Major components of hemostasis
In video
Vascular injury
⬇️ collagen + tissue factor
Vasoconstriction
Platelet activation
Coagulation Cascade
Antithrombotic control mechanism
Slide 4
Defective hemostasis bleeding
- no vessel constriction
- no platelet plug
- no fibrin clot
Coagulation cascade
Slide 7
Where can the problem be?
Blood vessels
- not intact
- inflam
- not normal
- eg Vaculitis (Henoch Schönlein purpura)
Platelets
- no platelet plug = bleeding
- 1. Decreased number (immune thrombocytopenic purpura -> not idiopathic but immune mediated)
- 2. Abnormal function (Glanzmann Thrombasthenia)
Clotting factors
- no fibrin clot = bleeding
- 1. Deficient (Haemophila A & B)
Blood vessels
Vasculitis
➢Inflammation of blood vessels
- e.g. Henoch Schönlein Purpura
- IgA mediated vasculitis of small vessels
- Triad = purpura/arthritis/abdominal pain
- Palpable purpura
- Typically lower limbs & buttocks
- Occasionally GIT bleed
- IgA nephropathy: proteinuria
Platelets
- Superficial bleeding
- Mucocutaneous bleeding pattern:
➡️Epistaxis, gingival bleeding, haematuria,
➡️Petechiae, purpura, ecchymoses - May stop with pressure
- never has haemarthroses
Clotting factors
- Most often after trauma
• Can be spontaneous as well (severely decreased levels) - Deep bleeding pattern:
• Haematomas
➡️Bleed into deep tissues: muscles
• Haemarthroses (classic)
➡️Bleed in joint - Can also have epistaxis, haematuria, ecchymoses, gingival bleeding
- Pressure usually insufficient to stop bleeding
Coagulation disorders
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History: ask right questions
- Recent infections eg Viral infections (*ITP)
- Severity
- Frequency
- Triggers egtrauma
- Ask about the “potential bleeding points” in a child’s life:
• Haematomas/bleeding at birth (especially vit K)
• Bleeding from umbilical stump
• Haematoma after immunisations (rare)
• Prolonged bleeding with teething - Any bleeding with previous surgery/procedures
History: age
- Age at start of symptoms
- Haemorrhage in neonatal period – suspect congenital cause strongly In older patient congenital or acquired
Infancy
- CONGENITAL
- Neonatal intracranial haemorrhage due to Vitamin K deficiency
- Post circumcision
- Umbilical stump bleeding : Factor XIII deficiency, Fibrinogen deficiency
- Wiskott Aldrich syndrome: X-linked recessive immunodeficiency disorder: thrombocytopenia, eczema, recurrent infections
- Start bearing weight→inherited coagulation disorders eg Haemophilia
Older child
- CONGENITAL
- Von Willebrand Disease= Most common congenital bleeding disorder!
-Inherited coagulation disorders eg Haemophilia A (A more common than B), Haemophilia B
- Platelet dysfunction (rare) eg Glanzmann Thrombocytopenia, Bernard Soulier Syndrome
History: gender
Boys
- X-linked recessive
- eg Haemophilia A
- Haemophilia B
- Wiskott Aldrich Syndrome
Boys and girls
- Autosomal recessive/dominant: Von Willebrand Disease (VWD)
- Autosomal recessive: Haemophilia C
Acquired:
- Immune Thombocytopenic Purpura (ITP)
- Disseminated Intravascular Coagulopathy (DIC)
Ethnicity
- Jewish population more common : Haemophilia C
- Clotting factor XI deficiency
- Autosomal recessive
- Boys and girls
History: family history
- Males with bleeding/ haemarthroses/ death
- Mother/female relatives:
➡️postpartum bleeding, need for blood transfusion, iron supplementation, menorrhagia
➡️Know how to identify menorrhagia - Inheritance patterns
- Spontaneous mutations
Screening tool for ID women with menorrhagia for testing and evaluation for underlying bleeding disorders
- duration of menses was greater than or equal to 7 days
- flooding or bleeding through a tampon or napkin in 2 hrs or less with most periods
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What bleeding pattern is present?
Mucocutaneous (superficial)
- = Platelets/ Von Willebrand types 1 & 2
- Petechiae
- Purpura
- Ecchymoses
- Mouth/nose bleeds
- Haematuria
- Rarely intracranial
Clotting factor deficiency (deep)
- = Clotting factor deficiencies / Von Willebrand type 3
- Haematomas (Soft tissue/muscle)
- Haemarthroses
- Ecchymoses
- Mouth/nose bleeds
- Haematuria
- Intracranial
- GIT
Slides 21 +22
Other history taking
Medication that may cause bleeding
- Aspirin
- Warfarin
- Non Steroid Anti inflammatory Medication eg Ibuprofen, diclofenac
Diet
- Vitamin K (intestinal flora, green leavy food) deficiency
- Vitamin C deficiency (scurvy) Perifollicular haemorrhage
Medic Alert
Underlying disease
- eg acute or chronic liver disease
Clinical examination
Observations
- Fever, tachycardia, tachypnea, hypotension
Anthropometry
- Fanconi anaemia – 75% short stature
Pallor
- Blood loss
- Part of bone marrow failure
- Haemolysis
Lymphadenopathy
Dysmorphic features
Skin:
- petechiae, ecchymoses, vasculitis, café au lait spots, blueberry muffin nodules, other masses/nodules
Abdomen
- Hepatosplenomegaly
- Abdominal mass (neuroblastoma, rhabdomyosarcoma, lymphoma)
CVS
- If pallor present, is the child symptomatic of the anaemia
- Most sensitive sign = resting tachycardia in the absence of fever, NOT a murmur!
Resp
- Respiratory distress
- Signs of superior vena cava syndrome
- Pleural effusion
- Consolidation/mass
CNS
- Level of consciousness
- Meningism
- Focal signs
Child with bleeding clinical examination
Bleeding symptoms and signs:
- Mucocutaneous bleeding: petechiae, purpura, ecchimoses
- Soft tissue bleeds: Haematoma
- Joint bleeds: Haemarthrosis
- Other symptoms and signs:
- Fatigue
- Weight Loss
- Pallor
- Lymphadenopathy
- Hepatosplenomegaly
Congenital bleeding disorder
Common ways of presentation
Known family history
- But family members do not always disclose
- Usually autosomal recessive; X-linked but may also be autosomal dominant (VWD)
- NO family history does not exclude congenital cause (spontaneous mutation)
Neonatal period:
- Intracranial or other sites of bleeding at birth
- Haematoma after IM (intramuscular) vitamin K
- Bleeding from umbilical stump
- Excessive bleeding after circumcision in 1st week of life
Haematoma after vaccinations (uncommon)
Easy bruising (parents sometimes accused of abuse)
Poor wound healing (uncommon)
Bleeding episodes
- Epistaxis
- Gingival bleeding
- Haematuria
- Haematomas
- Haemarthroses
Excessive bleeding after trauma or procedures
- Tear of tongue frenulum leads to ++bleeding (eg when drinking bottle)
- Dental or other procedures
Von Willebrand disease
- Most common inherited bleeding disorder
- Mucosal bleeds, Epistaxis
- Boys and girls
- Older child, adolescent ,post surgery/procedure bleeding
- *Type 3 may behave like Haemophilia
Haemophilia A & B
- Inherited, X-linked recessive
- Spontaneous mutations
- Males (females extremely rare)
- Haemophilia A = Clotting factor VIII deficiency (more common)
- Haemophilia B= Clotting factor IX deficiency
(Christmas disease) - Haemarthrosis = bleeding in joint
- Haematomas=bleeding in soft tissue
- Can also have mucosal bleeding eg Haematuria,epistaxis
- Inhibitors (measured in Bethesda units)
➡️Inhibitors are neutralising antibodies against the deficient clotting factor that limit the effectiveness of Factor infusions
Common ways of presentation of an acquired bleeding disorder
DIC/ Malignancy/ Other
- Acutely ill/unwell child
- Infection
- Petechiae/purpura
- Ecchymoses
- Bleeding from venipuncture sites
- Bleeding from mouth/nose/GIT/other sites
- Ischaemia (peripheral)
- Lymphadenopathy
- Hepatosplenomegaly
- Other
Immune Thrombocytopenic Purpura
- Well child
- Petechiae and purpura
- Ecchymoses
- Bleeding from mouth/nose
- Less commonly other sites
- Often following recent (2-3 weeks prior to rash) viral infection or (rarely) vaccination
- NO lymphadenopathy
- NO hepatosplenomegaly
Immune thrombocytopenic purpura
- Most common thrombocytopenia in children
- Age:2-5years
- Boys and girls
- Well child
- Post viral infection (2-3 weeks prior to rash)
- Petechiae, purpura, ecchymoses
- Epistaxis,oralbleeding
- Severe bleeding eg intracranial bleeding uncommon (<1%)
- NO lymphadenopathy
- NO hepatosplenomegaly
Important
- Patients with clotting factor deficiencies can have haematuria, epistaxis and mouth bleeds, but NOT petechiae/purpura
- Patients with platelet disorders DO NOT get muscle/joint bleeds
- Patients with VWD can have superficial and/or deep bleeds depending on subtype
Disseminated intravascular coagulopathy (DIC)
- Acquired Bleeding disorder
- Complication of Underlying conditions eg
• Septicemia
• Viraemia
• Shock
• Hypoxia
• Burns
• Malignancy (Acute Promyelocytic Leukaemia)
• Post operative
Pathogenesis Slide 39
Vit K deficiency
Acquired bleeding disorder
Vitamin K
- Required by liver for activation of Factors II, VII,
IX, X
9 (+1) = 10)
( To remember: 2 + • Natural K vitamins synthesised by Intestinal flora
7 =
• Fat soluble, require bile for absorption, not stored in body
Vit K deficiency
Vitamin K deficiency
• liver synthesises abnormal proteins nl PIVKAs, which are inactive and may cause inhibition of coagulation Neonate at risk.
Other causes: obstructive jaundice, malabsorption syndromes, broadntibiotics long tem use,oral anticoagulants , diet deficient aan vitK
Haemorrhagic disease of newborn (HDN)
Early HDN: Bleeding hours to 1 week after birth : due to Maternal medication eg Anti-convulsant Classic HDN : Bleeding 1-3 weeks : due to missed Vit K at birth
LateHDN :Bleeding1-3monthsoflife (higherriskofintracranialbleeding)
Clinical: melaena, haematemesis, umbilical cord bleeding
Liver disease
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