Practical approach to a bleeding

child
BY
Dr :- Hisham Mustafa Alomda
Assistant Professor of Pediatrics, Gezira University
MD , Pediatrics & Child health U of K
 NORMAL CLOTTING
Response to vessel injury
1. Vasoconstriction to reduce blood flow
2. Platelet plug formation (von willebrand factor
binds damaged vessel and platelets)
3. Activation of clotting cascade with
generation of fibrin clot formation
4. Fibrinolysis (clot breakdown)
 PRACTICAL APPROACH TO A CHILD WITH BLEEDING
HISTORY

• HISTORY – HISTORY – HISTORY

>Age of onset
> Sex
>Frequency
>location / type of bleeding
>Duration of bleeding
> Medications
> Associated symptoms
> Review of systems
Approach to a bleeding patient
–What is the type of bleeding disorder?

Primary hemostasis :
Vascular causes
Platelets-Number vs. Function
Fibrin formation : clotting factors

Premature clot dissolution: post clot formation

Approach to a bleeding patient
–Is a bleeding tendency present?

Easy Bruising
Severity of trauma
Mucosal bleeding
Menorrhagia
Surgical Hemorrhage
Joint and Muscle bleed
Approach to a bleeding patient
Is the disorder Familial or Acquired?

Family history – MOTHER & OTHER

FEMALE MEMBERS
• Vascular causes –
Infectious causes – “Meningococcemia”

Vasculitis – Henoch-Schonlein Purpura

 PRACTICAL APPROACH TO A CHILD WITH BLEEDING

• PHYSICAL EXAMINATION-
> Peticheae
>Echymoses
>Joint bleed &deepseated hematomas
> Hepatosplenomegaly
>Significant lymphadenopathy
> Active and playful vs. Ill looking
> Dysmorphic features
>Hemangiomas
Ecchymosis
 Primary Hemostasis Secondary Hemostasis

Onset Immediate Delayed

Sites Superficial Deep
Skin Petechiae, superficial Deep ecchymosis,
ecchymosis hematoma

Mucosal Common Rare

Others Rare Retroperitoneal

hematoma, hemarthrosis
 PRACTICAL APPROACH TO A CHILD WITH BLEEDING
• LABORATORY WORK UP-
P.M.D -
> C.B.C./PLATELET COUNT
>Peripheral smear- morphology
> P.T. [Prothrombin time] (extrinsic &common pathways )
> a.P.T.T. [ Activated partial thromboplastin time](intrinsic
and common pathways )
> T.T. [Thrombin time] common pathway
> Bleeding time
>Platelet aggregation studies(platelet function)
> Factor assay
Platelet Disorders
 Bleeding disorders
• Platelets–

Acquired causes much more common

Thrombocytopenia more common than
functional defects
Inherited disorders – both number &functional defects
are extremely rare
Disc shape cells 2-4 mm in diameter.
They are formed in BM from their progenitors the
megakaryocytes.
Their normal count in peripheral blood is 150000-
450000/µL.
Their life span is 9-10 days.
 PLT function
Adhesion to blood vessel wall,
Aggregation to form the primary haemostatic
plug at site of blood vessel injury, &
Release of vasoconstrictor substances from
intracellular granules in response to variety
of substances are the main functions of
platelets.
THROMBOCYTOPENIA

• Decreased production
• Increased destruction
• Sequestration
DECREASED PLATELET PRODUCTION

• Marrow failure (pancytopenia)

▪ A plastic anemia, chemotherapy, toxins
• B-12, folate
• Viral infection
• Drugs that can selectively reduce platelet production
▪ Alcohol
▪ Estrogens
▪ Chlorpropamide
▪ Interferon
• Inherited thrombocytopenia
• Congenital a plastic anemia/Fanconi's anemia
INCREASED PLATELET CONSUMPTION

• Immune destruction(ITP)
• Intravascular coagulation (DIC -HUS)
• Damage by bacterial enzymes, etc
Idiopathic Thrombocytopenic Purpura
(ITP)
It is a bleeding disorder caused by autoimmune
Abs destroying patient's own platelets by
phagocytosis in the spleen (to a lesser
degree in the liver).
Usually proceeded by upper respiratory tract
infection
 Epidemiology
*Children are affected by an acute type of illness
following URT infection (usually viral).
*♂ = ♀ incidence.
*Adults are affected by a more gradual onset of
disease with chronic course
 Clinical features and diagnosis
• Petechiae, purpura,ecchimosis
• Mucosal bleeding( bleeding gum, epistaxis,
menorrhagia, GI bleeding & hematuria)
• Major internal/intracranial bleeding rare
• Mortality rate < 5%
• Absence of constitutional symptoms or splenomegaly
 Diagnosis
CBC
Platelet count < normal (Thrombocytopenia)
* If > 100000/µL no spontaneous bleeding even with major surgery
*If 50000-100000/µL Bleeding more than normal with severe
trauma
*If 20000-50000/µL Bleeding occurs with minor trauma
*If < 20000/µL spontaneous bleeding occurs
Hb and TWCs are normal
Other blood counts and coagulation parameters
normal
• Marrow shows increased megakaryocytes .
 Confirmatory laboratory testing

• Serum anti-platelet antibody assay (poor

sensitivity)

• Test for specific anti-platelet glycoprotein

antibodies)
Confirmatory testing not necessary in typical cases
 Management
Children have a self-limited disease & 70% recover in 4-6
weeks.
• Corticosteroids:
• Mechanism of action: Slows platelet destruction,
reduces autoantibody production
• Prednisone, 1-2 mg/kg/day (single daily dose)
• Begin slow taper after 2-4 weeks (if patient responds)
• Consider alternative therapy if no response within 3-4
weeks
• About 2/3 of patients respond (plts > 50000) within 1
week
• Intravenous immunoglobulin therapy
• Possible mechanisms of action:
▪ Slowed platelet consumption by Fc receptor
blockade
▪ Accelerated autoantibody catabolism
▪ Reduced autoantibody production
• Dose: 0.4 g/kg/d x 5 days (alternative: 1 g/kg/d x
2 days)
– About 75% response rate, usually within a few
days to a week
– Over 75% of responders return to pre-treatment
levels within a month
• Splenectomy
 von Willebrand Disease
• The most common inherited bleeding disorder
• Inherited as AD
• Occurs in 1% of the population
• Less than 10% of patients have bleeding
events due to vWD
 Functions of vWF
• Binds to platelet receptor GP Ib and to subendothelial
structures such as collagen serving as bridge between
platelets and subendothelium in damaged vessels
• Acts as bridge between adjacent platelets in vessels
• Binds to circulating factor VIII protecting it and
prolonging FVIII t1/2 in the circulation from 2 to 8-
12 hours
 Symptoms of vWD
• Easy bruisability
• Epistaxis or gingival bleeding
• Menorrhagia
• Post-partum hemorrhage
• Post-surgical bleeding
• Bleeding post-dental extraction
 Classification of vWD
Sub types of VW

Type 1 Partial quantitative deficiency of vWF

Type 2 Qualitative variants of vWF

Type 3 Complete deficiencey of vWF & Behave as Severe

Hemophilia A
 Investigation
• PTT : increase
• PT : normal
• Bleeding time : increase
Treatment Guidelines in VWD
TYPE TREATMENT
1 DDAVP/FVIII-VWF

2 DDAVP/FVIII-VWF

3 FVIII-VWF
Hemophilia
 Hemophilia
• Caused by an absence or decreased
amount of a procoagulant –
• VIII -Hemophilia A X-R
– affects ~ 1:5000 males
• IX -Hemophilia B X-R
– affects ~ 1:30000 males
• XI –Hemophilia C AR
– Rare
 Epidemiology

Incidence: Hemophilia A - 1:5,000

Hempohilia B – 1: 30, 000

Hemophili Hemophilia C
a A

Hemophili
a B
Inheritance
Inheritance
 HEMOPHILIA SEVERITY LEVELS

Clinical manifestations F actor activity Severity

Spontaneous <1% Severe

hemorrhage from early
infancy
Freq sp hemarthrosis

Hemorrhage sec to 2-5% Moderate

trauma or surgery
Occ sp hemarthrosis

Hemorrhage sec to >5% Mild

trauma or surgery
Rare sp bleeding
 Clinical Manifestations: Hemarthrosis
• The most common, painful and most
physically, economically and
psychologically debilitating manifestation.
• Bleeding probably starts from synovial
vessels into the synovial space.
• Clinically:
➢ Pain
➢ Generally affects one joint at the time
➢ Most commonly: knee; but there are others
as elbows, wrists and ankles.
➢ Edema, erythema, warmth
➢ There is destruction of surrounding
structures as well-bone necrosis and cyst
formations,
➢ Complications: Chronic involvement with
joint deformity complicated by muscle
atrophy and soft tissue contractures
 Hematomas

• Subcutaneous and muscular hematomas spread

within fascial spaces, dissecting deeper structures
• May compress vital structures: such as the airway if
it is bleeding into the tongue throat or neck; it can
compromise arteries causing gangrene and ischemic
contractures are common sequelae, especially of
calves and forearms
• Muscle hematomas: 1)calf, 2)thigh, 3)buttocks,
• Retroperitoneal hematoma: can dissect through the
diaphragm into the chest compromising the airway.
It can also compromise the renal function if it
compresses the ureter
Intracranial hemorrhage
• Leading cause of death of
hemophiliacs
• Spontaneous or following
trauma
• May be subdural, epidural
or intracerebral
• Suspect always in
hemophilic patient that
presents with unusual
headache
• If suspected- FIRST
TREAT, then pursue
diagnostic workup
 Clinical manifestations
Others
• Gastrointestinal Bleeding:
• Mucous Bleeding:
Epistaxis, gum bleeding.
• Genitourinary Bleeding:
Frequently severe hemophiliac can experience
hematuria and a structural lesion should be ruled out.
 Investigations
• PTT : prolonged
• PT : normal
• Factor assay to confirm the diagnosis
 Factor replacement
• 1 u/kg raises FVIII levels 2%
1/2 life : 12 hrs

• 1 u/kg raises FIX levels 1 %

1/2 life 20-24 hrs
 Minor Bleeding Episodes
• Early joint bleeds
• Soft tissue & muscle bleeds
• Nose & gum bleeding not responding to
local measures
• Treatment of minor bleeding episodes
– 40 - 50% correction
– FVIII : 25 units / kg
– FIX : 50 units / kg
 Major Bleeding Episodes
• Advanced soft tissue & muscle bleeds
• Head & neck injuries
• Gastrointestinal bleeding
• Advanced joint bleeding
• Treatment of major bleeding episodes
• 80 – 100 % correction
• FVIII : 50 units / kg
• FIX : 100 units / kg
 Current Products
• Plasma Products: plasma-derived factor VIII concentrate
• DDAVP:
– Causes release of factor VIII/vWF
– Increased factor activity in 30-60”
– For mild hemophiliacs and mild bleeding symptoms
 INHIBITORS

30% of people with haemophilia develop an antibody to

the clotting factor they are receiving for treatment.
These antibodies are known as inhibitors.
These patients are treated with high does of FVIIa for
bleeds or surgery. This overrides defect in FVIII or FIX
deficiency.
Long term management involves attempting to eradicate
inhibitors by administering high dose FVIII (or FIX) in a
process called immune tolerance
Clinical Severity
 Hemophilia Treatment Center Team
Members

• Patient / Family • Primary Care

• Hematologist • Infectious Disease
• Nurse • Genetics
• Social Worker • Pharmacy
• Physical Therapist • Dentist
• Orthopedist • Hepatologist
 Hemorrhagic disease of the newborn

• A moderate decrease in factors II ,VII ,IX ,X

normally occurs in all newborn infants by 48-72hours
after birth ,this is probably due to lack of free VitK
from the mother and absence of bacterial intestinal
flora normally responsible for VitK synthesis
• The most common sites of hemorrhage or
bleeding are : gastrointestinal, nasal ,
subgaleal, intracranial,or postcircumcision
 Types:
1. Classic : occurs in the first 48-72hours
2. Early onset : very rare, 0-24 hr, maternal drugs:
phenobarbital, phenytoin, warfarin, rifampin, INH; they
interfere with VitK
3.Late –onset vitamin K deficiency
(1-6mo)bleedinginclude:
•Malabsorption
•Hepatitis , biliary atresia
•Cystic fibrosis
•Alpha1-antitrypindeficiency
• Investigations:
– PT,PTT are prolonged
– The levels of factors II, VII, XI, X are low
• Prevention of vitamin K deficiency bleeding
with intramuscular vitamin K is of primary
importance in the medical care of neonates . A
single dose of intramuscular vitamin K after
birth effectively prevents classic vitamin K
deficiency bleeding particularly in term infants
 Treatment
• Intravenous vit K
• FFP
• Blood transfusion
 Factor XIII deficiency
• AR
• Stabilizing the clot
• Patient present with delayed bleeding
• Decrease level of factor XIII
• Treated by factor replacement
Thank you