BLEEDING DISORDERS
Dr Kailash Prasad Sah
MBBS(Cal) DCH ,MD(Ped)
Hemato-Oncology( UK,DELHI)
Associate Prof.
BLEEDING DISORDERS
1) Platelets disorders
2) Coagulation disorders
3) Vascular disorders
BLEEDING DISORDERS
Common Causes like :-
Coagulation disorders like-
Hemophilia A& B,
vWD, ,Hypofibrogenemia.
Platelets disorders like:-
ITP,
Functional disorders – Bernard –Soulier
Syndrome
Glanzman thrombasthenia. And Gray
platelets Syndrome.
Vascular- HUS,HSP,Vit.C deficiency and
drugs.
BLEEDING DISORDER
Physiology of Hemostasis
Whenever a blood vessel is cut or rupture , the bleeding
usually stops spontaneously after some time by the
following mechanisms.
i)Vascular spasm
- The blood vessels contract as a result of neurological
reflexes and local myogenic spasm.
- Spasm last for 20 to 30 minutes.
ii) Platelet plug formation.
Platelets come in contacts the subendothelial matrix
become irregular and sticky .
VWF receptor binds.
After adherence.
Platelet liberate ADP, thromboxane and serotonin, which
in return activate more platelets.
They become sticky and aggregate together to form a loose
platelet plug.
iii) Plasma phase(clotting)
- Blood clots develop within a minute or too.
- Traumatized vessels and platelets liberate activating
substances which initiate the clotting process.
Physiology of coagulation pathway
BLEEDING Pattern -
Coagulation / Platelets disorder
Platelets- Skin,Mucous
membrane ,Petechial Rash,
minor trauma- small superficial echymosis
( ITP ,vWD)
Coagulation- Soft Tissue bleed and
joint .Echymosis is large due to accident.
( Hemophilia)
IMMUNE THROMBOCYTOPENIC
PURPURA(ITP)
Characterized by
-Petechial hemorrhages.
- Ecchymosis.
- Thrombocytopenia.
- Reduced platelet survival.
- Presence of platelet antibodies.
- Normal or increased number of megakaryocytes
in the bone marrow.
PATHOGENESIS
Thrombocytopenia results from the increased destruction
of the antibody coated platelets by the reticuloendothelial
system in the spleen and liver.
Classification of ITP:
Acute ITP.
Chronic ITP.
Acute ITP:
Sudden onset.
Preceded by viral infection.
Duration- 4-6 weeks.
Incidence-2-6 yrs.
Severe thrombocytopenia.
80% cases resolve spontaneously within 1-2 months.
Chronic ITP:
Insidious onset.
Persistent thrombocytopenia for at least six months.
Only 10-20% chance of spontaneous remission.
More common in girls.
Clinical features:
Easily bruisability.
Subcutaneous hemorrhage.
Petechiae.
Ecchymoses-anterior surface of lower limb and over bony
prominence.
Bleeding from mucosal surface.
Hematemesis, melena and bleeding in the joints are
unusual .
Intracranial bleed.
Anemia.
Spleen- may or may not be palpable.
Severe manifestation:
Hematuria.
Gastrointestinal bleeding.
Nasal Bleed(Epistaxis)
ITP
Severe epistaxis.
Menorrhagia.
Intracranial bleed.
Diagnosis:
Investigation – CBC and PBS . Plaletels morphology.
Platelet count - <1,00,000/mm3.
Bleeding time – prolong.
P.T – normal.
A.P.T.T – normal.
Bone marrow-normal or increased number of
megakaryocytes.
Treatment:
No specific therapy-if asymptomatic with platelet >
40,000/mm3.
Supportive care
- Restriction of physical activity.
- Avoidence of aspirin and related drugs.
- I.M injection should be avoided.
Should be admitted if platelet < 20, 000 /mm3.
CORTICOSTEROIDS
Prednisolone.
1-2mg/kg/day for 2-3 weeks and tappering over 1-2 week.
Methyl prednisolone-30mg/kg/day for 3days.
-Inhibit platelet antibody production.
-Inhibit interaction between the platelet and antibodies.
-Prolong platelet survival.
-Improve vascular stability.
INTRAVENOUS
IMMUNOGLOBULIN
2gm/kg (total dose)
i) 0.4 gm/kg 0.D* 5 days.
ii) 1gm/kg 0.D * 2 days.
Increase the platelet count by blocking the Fc receptor and
protect the platelet from antibodies.
Anti-Rh (D) Therapy:
25microgm/kg * 3 days.
Blockade the Fc receptor by the antibody coated RBC in
place of antibody coated platelets.
SPLEENECTOMY
Chronic ITP.
Uncontrolled bleeding or those not responding to steroids
or IVIG therapy.
Treatment of chronic ITP:
Corticosteroids
- Initiated as in acute ITP and subsequently small dose
daily or on alternate day for 4-6 months.
Immunoglobulin
- I.V-IgG (0.4-1gm/kg/every 2-6 weeks.)
PLATELET FUNCTIONAL
DISORDERS
i)Glanzmann’s thrombasthenia.
-A.R, congenital disorder.
- Platelet receptor GP IIb/IIIa absent.
- Prolong bleeding time.
- Normal platelet count.
ii)Storage pool disease.
- Dense granules are reduced.
iii)Gray platelet syndrome
- Absence of platelet alpha granules.
iv)Bernard soulier syndrome.
- Absence or severe deficiency of the VWF receptor
(GPIB) complex on the platelet membrane.
Characterized by- A.R
- Thrombocytopenia.
- Giant platelets.
- Prolong bleeding time.
v) Wiskott-Aldrich Syndrome
X- linked recessive inherited disorder.
Characterized by triad of
- Eczematoid dermatitis.
- Immunodeficiency.
- Thrombocytopenia.
TREATMENT
- Desmopressin, 0.3microgm/kg-( mild –moderate)
-Platelet transfusion
-stem cell transplantation - curative
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