BLEEDING DISORDER

IN
CHILDREN
Normal haemostasis:- Haemostasis is an active process that clot the
blood in the area of blood vessels injury and yet simultaneously limit
the clot size to the area of injury. Over the time the clot is lysed by
fibrinolytic system, and normal blood flow is restored.
Platelets is the main key factor that first adhere the site of injury and
provides a reaction surface where the clotting factors binds and the
coagulation cascade start. Active clotting is controlled by negative
feedback loops that inhibit the clotting process when procoagulent
process comes in contact with intact endothelium.
Main components of haemostasis process are – Vessels wall, Platelets,
Coagulation proteins, Anticoagulent factors and Fibrinolytic system.
 Terms used in clinical manifestation

• Petechiae: Bleeding spots that are pin head size

measure 3 mm or less.
• Purpura:- The spots are caused by bleeding
underneath the skin, purple in colour and size is 0.3-
1 cm. they doesn't bleach on pressure.
• Ecchymosis: - Bleeding spots that measure more
than 1 cm.
 Common causes of bleeding in children.
• Idiopathic thrombocytopenic purpura (ITP). Most
common.
• Haemophilia A, B and C.
• Aplastic anaemia.
• Von Willebrand disease
• Leukaemia
• Henoch Schonlein purpura.
• Dengue haemorrhagic fever.
• Other:- Glanzmann disease, Bernard Soulier
syndrome, DIC etc.
 Idiopathic Thrombocytopenic Purpura (ITP)
• This is the most common bleeding disorder in
children.
• In this condition are platelets are coated with
circulating antibody (mostly viral origin). For this they
lost their functions and destroyed in spleen.
• In most of the cases there is a history of viral
infection ( upper respiratory tract infection) about 2-
4 weeks before the onset of bleeding manifestation.
The viruses are Epstein Barr virus, Rubella, Varicella,
Measles, Parvovirus and Influenza virus.
• In most of the cases there is natural remission within
2 months.
 Classification of ITP

• Acute ITP
• Chronic or Persistent ITP. Where thrombocytopenia
persist more than 12 months.
 Acute ITP

• Occurs in children 1-4 yrs.

• Sudden onset of generalized petechiae and purpura
(bruise and purple dots) in a previously healthy child.
• There may be bleeding from gum, mucus membrane,
conjunctiva and nose ( Epistaxis).
• Some times there may be internal bleeding like
haematuria, Haematemesis and intracranial bleeding
when there is severe thrombocytopenia and may be
fatal.
 New United Kingdom classification on the basis of
sign and symptoms.

• No symptoms.
• Mild symptoms- Bruising and petechiae, occasional
mild epistaxis and very little interference with daily
living.
• Moderate- more severe skin and mucosal bleeding,
troublesome epistaxis and menorrhagia.
• Severe- bleeding episode like menorrhagia–
epistaxis– melena requiring transfusion and
hospitalization.
 Physical examination
• Skin – Petechiae, Purpura and ecchymosis.
• Mucus- bleeding from nose , gum, oral cavity and conjunctiva.
• Patient neither have severe pallor, Hepatosplenomegaly,
Lymphadenopathy and nor bony tenderness.
 Investigation or Laboratory finding
• In acute ITP- Haemoglobin level, WBC count and differential
count of WBC are normal. Hb. May low in severe bleeding.
Platelets count may be normal or severely decrease(
Thrombocytopenia < 20X109/l.) and sometimes large platelets
may seen.
• Coagulation profile- Bleeding time (BT) prolong, but
Coagulation time (CT) (usually not done), Prothrombin time
(PT) and Activated partial thromboplastin time test (aPTT) are
normal.
• Bone marrow:- Normal granulocytic and erythrocytic series
with characteristically normal or increased number of
megakaryocytes. Bone marrow is not routinely done and is
indicated when there is abnormal WBC count, unexplained
anaemia, Organomegally or lymphadenopathy.
• Platelets antibody test- use rarely.
 Differential diagnosis

• Henoch Schonlein purpura

• Dengue haemorrhagic fever
• Aplastic anaemia
• Leukemia.
• Meningococcal septicemia
• others
 Treatment
• Most of the ITP is self limiting and about 80% require no
therapy unless counseling. However treatment is required
when platelets count fall < 20,000/dl.
• Treatment option are:-
– Intravenous immunoglobulin (IVIG) – 0.8-1gm/kg iv for 2 days.
– Oral prednisolone (1-4mg./kg/day ) for 2-3 weeks platelets count
rises more than >20,000/dl. And rapid tapering within a week.
– Methylprednisolone (30 mg./kg) iv for 3 days.
– Anti-D immunoglobulin (60-75ugm./kg) iv single dose in Rh-
positive patient.
– Splenectomy.
– Platelets transfusion
 Indication of splenectomy

• Older children with Chronic ITP.

• Life threatening condition like intracranial hge. where
other measure fails.
 Chronic ITP

• About 20% of patient with acute ITP have persistent

thrombocytopenia for more than 12 months and are
called chronic ITP.
• Treatment is initially same as acute ITP but may fail
or presented with complication. In this cases treated
with RITIXIMAB. Or Splenectomy. As the spleen is the
main site of anti platelets antibody production and
destruction. Success rate splenectomy is 64- 88%.