Bleeding in children

1. Tutorial 10: Bleeding disorders
in children
 Physiology of haemostasis &
coagulation pathways
 Common bleeding disorders

2. Haemostasis of Blood
1. Vascular spasm and vasoconstriction
2. Formation of platelets plug
3. Blood coagulation
4. Formation of fibrin mesh

5. Blood Coagulation

7.  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)

8.  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)

9.  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)

10. Bleeding disorder in
children
 Defects / abnormalities in :
1. Vascular OR
2. Platelets ( qualitative/quantitative)
OR
3. Coagulation

11. Bleeding Due to
Vascular Defect
Anaphylactoid
Purpura;
Henoch-
Schonlein
purpura
Infective states;
meningococca
emia & dengue
haemorrhaic
fever
Nutrition
Deficiency;
Vitamin C
(Scurvy)
Miscellaenous;
hereditary
haemorrhagic
telangiectasia,
polyarteritis
nodosa &Ehlers-
Danlos Syndrome

12. 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

13.  -Haemophilia
 -Von Willibrand Disease

14. -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

15. Signs and symptoms
Present 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

16. Diagnosis
 History and signs
 Activated partial thromboplastin time
(prolonged)
 Factor VIII assay <10 IU
 Prenatal test-genetic testing have been
made available

17. 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

18. Complications
• Deep internal bleeding
-Joint damage from haemarthrosis
-Intracranial haemorrhage
• Transfusion transmitted infection

19. -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

20. Signs and Symptoms
 Varying degree of easy bleeding
 Mucosal bleeding such as epitaxis and gum
bleeding
 Bruising
 Menorrhagia
 Severe internal or joint bleeding uncommon

21. 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.

22. 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

23. 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

24. Platelet Disorders
 Leads to defects in primary haemostatic
mechanism
 Increased bleeding time
 Purpura and petechiae

25. Types of platelet disorders
 Qualitative (dysfunction)
 Glanzman disease
 Quantitative reduced (Thrombocytopenia)
 Decreased production: aplastic anemia, leukemia
 Increased destruction: ITP, drugs, collagen disorders
 Increased consumption: DIC, septicaemia

26. IMMUNE
THROMBOCYTOPENIA
Classified according to antigenic stimulus
 Autoimmune
 Immune/idiopathic thrombocytopenic purpura
(ITP)
 Alloimmune
 Posttransfusion purpura
 Neontal alloimmune thrombocytopenia
 Drug-induced
 Splenic destruction of antibody coated platelet

27. 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

28. 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

29. How to diagnose?
Low platelet count
No other blood abnormalities
Exclude 2⁰ causes: leukemia,
medications, lupus, cirrhosis,
HIV etc

30. Findings
No splenomegaly
Prolonged bleeding time
Bone marrow examination:
increased megakaryocytes

31.  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