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Bleeding in children
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Tutorial 10: Bleeding disorders in children
Physiology of haemostasis & coagulation pathways
Common bleeding disorders
Haemostasis of Blood
1. Vascular spasm and vasoconstriction
2. Formation of platelets plug
3. Blood coagulation
4. Formation of fibrin mesh
Blood Coagulation
Abnormal /normal bleeding? The onset of bleeding
- neonatal period (intrauterine or postnatal infection)
- 2nd to 5th day of life (hemorrhagic disease of newborn)
- Prolonged bleeding after trauma and dental extraction (congenital or acquired disorders)
The site of bleeding- Bleeding into joints or muscles (Hemophilia A & B)- The nose (epistaxis) mainly due to von-willebrand’s
disease- Abnormal bleeding involves gums, skin and under
periosteum of long bones (scurvy)
- GIT hemorrhage in neonatal period (hemorrhagic disease)
Clinical Approach To Diagnosis
The past history- Easy bruising, bruising at abnormal
sites, prolonged bleeding after trauma or invasive procedure
The family history- Hemophilia A, Christmas disease (sex-
linked recessive)- Von- Willebrand’ s disease &
hemorrhagic hereditary telangiectasia (autosomal dominant)
Drug ingestion (warfarin, heparin & aspirin) Diet ( vitamin C and Vitamin K) Splenomegaly (hypersplenism) – underlying causes like
leukemia Miscellaenous ( Wiskott-Aldrich Syndrome characterized
by eczema, thrombocytopenia and immunodeficiency & Ehlers-Danlos syndrome due to platelet aggregation failure)
Bleeding disorder in children
Defects / abnormalities in :
1. Vascular OR
2. Platelets ( qualitative/quantitative) OR
3. Coagulation
Bleeding Due to Vascular Defect
Anaphylactoid Purpura; Henoch-
Schonlein purpura
Infective states; meningococcaemia & dengue haemorrhaic
fever
Nutrition Deficiency; Vitamin C (Scurvy)
Miscellaenous; hereditary
haemorrhagic telangiectasia,
polyarteritis nodosa &Ehlers-Danlos Syndrome
Anaphylactoid purpura
Small vessel vasculitis by immune complexes
Combination of skin rashes (purpura), athralgia and abdominal pain.
Usually occur at age between 3-10 year old
Common in boys-preceded by pharyngitis
-Haemophilia -Von Willibrand Disease
Coagulation Disorder
HAEMOPHILIA-is a group of hereditary genetic disorders that impair the body's ability to control blood clotting or coagulation
Classification :
-Haemophilia A is a recessive X-linked genetic disorder involving a lack of functional clotting Factor VIII and represents (80%)
-Haemophilia B is a recessive X-linked genetic disorder involving a lack of functional clotting Factor IX. (20%)
-X-linked recessive,male predominance,qualitative/quantitative defect
Signs and symptomsPresent towards the end of first year of life(vary) Recurrent spontaneous bleeding into joints and muscles Large bruises and haematomas Internal or external bleedings
Three levels of hemophilia are recognized,
according to the level of clotting factor amounts in
the blood. These are often expressed as
percentages of normal: Above 5% - mild hemophilia 1% to 5% - moderate hemophilia Less than 1% - severe hemophilia
Diagnosis
History and signs Activated partial thromboplastin time
(prolonged) Factor VIII assay <10 IU Prenatal test-genetic testing have been
made available
Management• Regular infusions of recombinant FVIII or FIX
concentrates• IM injections, aspirin and NSAIDs and trauma
should be avoided in patients with haemophilia.
• Desmopressin (DDAVP) and cryoprecipitate for mild or moderate haemophilia A
• Specialised physiotherapy-pain,reabsorption and prevent/treat injury
Complications
• Deep internal bleeding
-Joint damage from haemarthrosis
-Intracranial haemorrhage
• Transfusion transmitted infection
VON WILLEBRAND DISEASE
-deficiency of von Willebrand factor. Von Willebrand factor facilitates platelet adhesion to damaged endothelium and also acts as the carrier protein for FVIII.
most common hereditary bleeding disorder, present in 1–2% of the general population.
Is usually inherited as an autosomal dominant trait and rarely as an autosomal recessive trait.
-Affects men and women equally
- Classification
Type 1- partial quantitative decrease of qualitatively normal von Willebrand factor
Type 2-qualitatively abnormal
Type 3-absent
Signs and Symptoms Varying degree of easy bleeding Mucosal bleeding such as epitaxis and gum
bleeding Bruising Menorrhagia Severe internal or joint bleeding uncommon
Diagnosis
• Bleeding time (is prolonged)
• APTT (prolonged)
• Ristocetin cofactor test (to measure vWF activity which uses the antibiotic ristocetin to induce vWF to bind to platelets. )
• von Willebrand factor level (level is reduced also act as APR)
Von Willebrand disease may be hard to diagnose.
Treatment
• DDAVP for milder form of vWD ( increase secretion of FVIII and vWD into plasma)
• Plasma derived FVIII concentrate for more severe types of vWD
• For women with heavy menstrual bleeding, the combined oral contraceptive pill may be effective in reducing bleeding or in reducing the length or frequency of periods
Contraindications Bleeding (hemorrhaging) may occur after
surgeries or other invasive procedures. If you have von Willebrand disease, do not
take nonsteroidal anti-inflammatory drugs (NSAIDs), such as aspirin or ibuprofen
Platelet Disorders
Leads to defects in primary haemostatic mechanism
Increased bleeding time Purpura and petechiae
Types of platelet disorders Qualitative (dysfunction)
Glanzman disease
Quantitative reduced (Thrombocytopenia)
Decreased production: aplastic anemia, leukemiaIncreased destruction: ITP, drugs, collagen disordersIncreased consumption: DIC, septicaemia
IMMUNE THROMBOCYTOPENIA
Classified according to antigenic stimulus Autoimmune
Immune/idiopathic thrombocytopenic purpura (ITP)
AlloimmunePosttransfusion purpuraNeontal alloimmune thrombocytopenia
Drug-induced Splenic destruction of antibody coated platelet
Acute ITP Affects young children (> 90% of cases) Acute in onset, usually self-limiting Majority of cases follow vaccinations or viral
infections ♂ : ♀ = 1:1 About 10% fail to recover within 6 months →
chronic ITP
Signs & Symptoms
<20,000/μl-admitted Spontaneous formation of purpura and
petechiae Epistaxis Bleeding gums Hematomas in mouth/mucous membranes
<5,000 /μl Subarachnoid/intracerebral hemorrhage Lower GI bleeding/internal bleeding
How to diagnose?
Low platelet count
No other blood abnormalities
Exclude 2 causes: leukemia, ⁰medications, lupus, cirrhosis, HIV etc
Findings
No splenomegaly
Prolonged bleeding time
Bone marrow examination: increased megakaryocytes
Full blood count Peripheral blood film Bleeding time (thrombocytopenia and dysfunction) Prothrombin time Activated Partial Thromboplastin Time
Coagulation factor assays (FXIII & FIX) aPTT & PT mixing studies Platelet funtion test & flow cytometry Thrombin time Liver & Renal Function Test D-dimers
Investigation of Bleeding