CHILD WITH

BLEEDING PROBLEMS
 EVALUATION OF A
BLEEDING CHILD
Normal bleeding
• Bruising and bleeding are commonly seen in children.
• Small bruises on bony prominences on front body are common place
• In non mobile infant <9m, significant bruising is unusual
• Epistaxis is also common in children but it usually stops within 2 minutes

Abnormal bruise / bleeding

• Uncommon sites for bruising at all ages are (back, buttocks, arms and abdomen)
• Epistaxis lasting >10 minutes
• ICB following birth trauma and bleeding from umbilical stump suggest a bleeding
disorder.
Important History
 The most useful initial screening tests are:
• Full blood count and blood film
• Prothrombin time (PT) – measures the activity of factors II, V, VII and X
• Activated partial thromboplastin time (APTT) –measures the activity of
factors II, V, VIII, IX, X, XI and XII
• Thrombin time – tests for deficiency or dysfunction of fibrinogen
• Biochemical screen, including renal and liver function tests.
Causes of Bleeding in children

Source from : Nelson’s Essential 5th Edition

 Thrombocytopenia
Thrombocytopenia is a platelet count <150 × 109/L.

The risk of bleeding depends on the level of the platelet count:

Severe thrombocytopenia (platelets <20 × 109/L)
– risk of spontaneous bleeding
Moderate thrombocytopenia (platelets 20–50 × 109/L)
– at risk of excess bleeding during operations or trauma but low
risk of spontaneous bleeding
• Mild thrombocytopenia (platelets 50–150 × 109/L)
-low risk of bleeding unless there is a major operation or severe
trauma.
Causes of Thrombocytopenia
Immune Thrombocytopenic Purpura (ITP)
*commonest cause of thrombocytopenia in childhood.

Acute ITP
• Benign self-limiting disorder, presenting with isolated thrombocytopenia (<100 x10^9/L), in the
absence of an underlying cause
• Diagnosis of exclusion
• Duration from onset of thrombocytopenia to normalization of platelet counts can be a few days to 6
months (average 3 weeks)
• 5% may have recurrence
• 10% may have Persistent / chronic ITP

Chronic ITP
• Persistent thrombocytopenia after 6 months of onset *(occurs in 20%)
Wide spectrum of manifestations:
• Mild : asymptomatic low platelet counts
• Intermittent : relapsing symptomatic thrombocytopenia
• Rare : persistent symptomatic and haemorrhagic disease
Signs and symptoms of Thrombocytopenia
Usually presents with
1. Bruising
2. Petechiae
3. Purpura
4. Mucosal bleeding (epistaxis or gum bleeding)

*major haemorrhage like severe GI bleeding, intracranial bleeding or

haematuria is less common
Feature Acute ITP Chronic ITP

Peak age Children (2-6 yrs) Adults (20-40 yrs)

Female:male 1:1 3:1

Antecedent Infection Common Rare

Onset of symptoms Abrupt Insidious

Platelet count at <20 000 <50 000

presentation

Duration 2-6 weeks Long term

Spontaneous remission Common Uncommon

Pathogenesis
 (IWG) Proposal On Classification Of ITP
Newly Diagnosed ITP Within 3 months from diagnosis

Persistent ITP Between 3 to 12 months from diagnosis. Includes patients not reaching spontaneous
remission or NOT maintaining complete response off therapy.

Chronic ITP Lasting for more than 12 months

Severe ITP Presence of bleeding symptoms at presentation sufficient to mandate treatment, or

occurrence of new bleeding symptoms requiring additional therapeutic intervention
with a different platelet-enhancing agent or increased dose.
Diagnosis & Investigation
• Diagnosis is based on history, PE, blood counts and FBP – diagnosis of
exclusion
• Physical examination
 Absence of hepatosplenomegaly or lymphadenopathy
• FBC:
 Isolated thrombocytopenia with normal hemoglobin and white cell count
• FBP
Normal apart from reduced, larger platelets, no abnormal cells

• Any atypical clinical features ( anaemia, neutropenia, hepatosplenomegaly marked

lymphadenopathy )  prompt a bone marrow examination to exclude acute
leukaemia or aplastic anaemia
• However, if the clinical features are characteristic, with no abnormality in the blood other
than a low platelet count  no need to examine the bone marrow.
Management
1. Most children remit spontaneously. Not all children with acute ITP need
hospitalization.
2. The platelet count is usually <20 x10^9/L at diagnosis
3. 70% achieve a platelet count >50 x10^9/L by the end of the 3rd week without
treatment.

Consider hospitalization in:

i) Severe life-threatening bleeding (e.g ICH) regardless of platelet count
ii) Platelet count <20 x10^9/L with evidence of bleeding
iii) Platelet count <20 x10^9/L without bleeding but inaccessible to healthcare
iv) Lack of confidence in homecare
Advise parent !
i) Precaution with physical activities especially small children
ii) Avoid contact sports
iii) Seek immediate medical attention if significant bleed
iv) Avoid aspirin /NSAIDs
v) Observation & monitoring of platelet count, without specific
treatment, is appropriate for patients with:
-Platelet count >20 x10^9/L without bleeding
-Platelet count >30 x10^9/L with only cutaneous purpura
-Repeat FBC within the first 7-10 days to ensure there is no evidence
of evolving marrow disorder
When to treat :
1) Life threatening bleeding episode (e.g ICH) regardless platelet count.
2) Platelet count <20 x10^9/L with mucosal bleeding
3) Platelet count <10 x10^9/L with any bleeding
Choice of treatment:
1) Oral Prednisolone 2mg/kg/day for 14 days -> then taper off over 5days
( REGARDLESS OF RESPONSE)
2) Oral Prednisolone 4mg/kg/day for 3-4 days
3) IVIG Immunoglobulin (IVIG) 0.8g/kg/dose for a single dose
* Side effects of IVIG (15-75%) : Fever, flushing, headache, nausea, aseptic
meningitis and possible transmission blood borne infection like Hepatitis C
WHAT is the MOST FEARED complication of
ITP ???
Intracranial Haemorrhage (ICH)
• Incidence in a child with ITP is between 0.1-0.5%
• The risk is highest with platelet count <20 x10^9/L, history of head
trauma, aspirin use and presence of cerebral arteriovenous
malformation.
• 50% of all ICH occurs after 1 month of presentation, 30% after 6
months.
• Early treatment with steroid or IVIG may not prevent late onset ICH.
Emergency Treatment of ITP with Severe
Bleeding
• severe epistaxis or gastrointestinal bleed, ICH
1. IV Methylprednisolone 30mg/kg/day for 3days
2. IVIG 0.8-1g/kg as a single dose- calculated to nearest bottle of IVIG
(usually 3 grams/bottle)
3. Combination of IVIG & methylprednisolone in life threatening
conditions.
4. Platelet transfusion in life threatening haemorrhage
5. Emergency splenectomy if other modalities fail
6. Neurosurgical intervention for ICH
Chronic ITP management
• Counselling and education of patient and caretakers regarding natural history of disease and
how to detect problems and possible complications
• Asymptomatic children: no need therapy, kept under observation and avoid contact sports.
• Symptomatic children : may need short course of treatments as for acute ITP during relapse
period or during surgical procedures.
• Revisit diagnosis to exclude other causes of thrombocytopenia (Immunodeficiency,
lymphoproliferative, collagen disorders, HIV infection).

*2nd line therapies Steroid pulses: oral Dexamethasone 1 mg/kg given on 4 consecutive days
every 4 weeks for 4 months.
TX of availability – Thrombopoietin receptor agonist, Rituximab, Splenectomy
https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6460888/#:~:text=The%2
0thrombopoietin%20receptor%20agonists%20(TPO,molecule%20agents%
HAEMOPHILIA
A group of blood disorders in which there is defect in
clotting factors
 70% are X-linked recessive disorder. 30%
spontaneous mutation.
 The bleeding patterns of haemophilia are similar.
The most common haemophilias are
1. Haemophilia A : Deficiency of Factor 8 ( 85%
cases)
2. Haemphilia B : Deficiency of Factor 9 ( 15% cases)
CLASSIFICATION
Classification Clinical Manifestation
•Manifest in infancy when child reaches toddler
stage
•Spontaneous bleeding – in muscles or joints
Severe (haemarthroses)
(<1% of normal) •Excessive bleeding after minor trauma,
postoperatively, or after intramuscular childhood
vaccinations.

• Manifest after 2 years of life

Moderate
• Moderate trauma causes bleeding episodes
(1-5% of normal)
• Occasionally spontaneous bleeding occurs

Mild • Often diagnosed in teenagers and adults

(>5% - <40% of • Significant trauma to induce bleeding
normal) • No spontaneous bleeding
 CLINICAL MANIFESTATION
 Haemarthrosis (spontaneous bleeding in muscle
or joints - painful)
 Iliopsoas bleeding
 Joint Swelling
 Easy bruising
 Epistaxis
 Haematuria
 Intracranial hemorrhage
- Bleeding during neonatal period is uncommon , usually presents when child
becomes mobile – crawling/ walking
- Bleeding may occur spontaneously or after trauma, operation ( circumcision)
or dental procedure
INVESTIGATION
 Full blood count
 Activated partial thromboplastin time (aPTT)
– PROLONGED
 Normal Prothrombin Time, Platelet Count,
Bleeding Time.
 Specific factor assay : VIII or IX - LOW
 Joint x-ray
 Further Investigation
 Hepatitis B, Hepatitis C, HIV serology- treatment carries risk
( checked yearly)
 Diagnosis for carrier status for genetic counseling
Treatment
Severe haemophilia should be prophylactic to prevent arthropathy and
ensure the best quality of life possible.
The dosage of prophylaxis
• 25-35 U/kg of Factor VIII concentrate  given every other day or 3 times
a week.
• 40-60 U/ kg of Factor XI  given every 2-3 days.
*However, this form of management is costly and requires central venous
access.
• Analgesia
• Good dental
hygiene
• Recombinant activated Factor VII (rfVIIa or Novoseven)
• FEIBA (factor eight inhibitor bypass activity)
VON WILLERBRAND
DISORDER
 Most common hereditary deficiency caused abnormality in von
Willerbrand protein.
 Functions on both primary & secondary homestasis.
 1. To act as bridge between subendothelial collagen and platelets
 2. Bind and protect factor VIII from rapid clearance then delivers it
to site of injury.

Types
CLINICAL
MENIFESTATION
 Asymptomatic

 Mucous membrane bleeding

 Epistaxis

 Cutaneus bleeding
 Gingival bleeding

 Menorrhagia
INVESTIGATION
 Full Blood Count – platelet normal
 aPTT PROLONGED or normal
 Factor VIII LOW or normal.
 von Willerbrand Factor activity (ristocetin cofactor)
 Ristocetin, an antibiotic that causes vWF to bind to platelet taken from
plasma.
 In healthy people, platelet rapidly agglutinate.

 von Willerbrand Factor antigen

 Measure vWF protein and binding sites.
Not accurate.
TREATMENT
 Desmopressin (DDAVP) – Treatment of choice for patients
with vWD types 1 and 2 .
 Concentrate of von Willerbrand Factor (Humarate P)
when high levels of vWF are needed but cannot achieved
with DDAVP (type 3)
 Contraceptive for menorrhagia
 Clot-stabilizing
medications (antifibrinolytic medications) -
PROGNOSIS &
COMPLICATIONS
 Lifelong
tendency toward easy bruising,
frequent epistaxis, and menorrhagia.
 Register with Malaysia Hemophilia Society.

 Carry medic-alert bracelet or chain & carry

books diagnosis, types etc.
VITAMIN K
DEFICIENCY
• Deficiency of coagulation factors II, VII, IX and X as a result of Vit K deficiency

Why decrease vitamin k in newborn?

• Lack of free vitamin K from the mother
• Absence of the bacterial intestinal flora which normally responsible for the
synthesis of vitamin K
• Breast milk is a poor source of vitamin K
3 main types of Vitamin K are
 K-1, phylloquinone, derived from plants;
 K-2, menaquinone, produced by the intestinal flora
 K-3, menadione which is a synthetic, water-soluble form used for treatment.

** Give parenteral vitamin K (0.5 to 1 mg) to all newborns shortly after birth
DISSEMINATED INTRAVASCULAR
COAGULATION (DIC)
“ Disorder characterized by coagulation pathway activation
leading to diffuse fibrin deposition in the microvasculature and
consumption of coagulation factors and platelets
Diagnosis
 The diagnosis of DIC usually suspected clinically and confirmed by a laboratory finding
of thrombocytopenia, prolonged PT and aPTT, low fibrinogen, raised fibrinogen
degradation products and D-Dimer and Microangiopathic Haemolytic anaemia
( MAHA)

 There is also marked reduction of naturally occurring anticoagulants, protein C, S and

antithrombin.

 In severely ill patient, there will be sudden occurrence of bleeding from venepuncture
or incision site, gastrointestinal or pulmonary hemorrhage, petechiae or ecchymosis or
evidence of peripheral gangrene or thrombosis.
TREATMENT
 Treat the underlying cause inducing the DIC
 Support the patient by correcting hypoxia, acidosis and poor
perfusion.
 Replace depleted blood clotting factors, platelets and
anticoagulant proteins by transfusion.
 Treatment with anticoagulants or coagulants contained in
fresh- frozen plasma usually needed in acute case.
 Drotregocin alfa (recombinant activated protein C) reduces
mortality in adults with DIC and sepsis ( severe
meningococcal septicaemia)
Reference
1- Paediatric Protocols 4th Edition
2- Illustrated Textbook of Paediatrics (Sunflower) 4th Edition
3- Textbook of Paediatrics and Child Health by Lee,Tay,Lum