2. • Bleeding may occur due to:
1)Defects in platelet
a)Thrombocytopenia
b)Qualitative disorders of function
2)Coagulation disorders
3)Dysfunctional fibrinolysis
3. CAUSES OF THROMBOCYTOPENIA
• ITP
• Infections: DIC, malaria, kala-azar, DHF, hepatitis B & C
• Medications : valproate, penicillin, heparin
• Thrombotic microangiopathy: TTP, HUS
• Malignancies: leukemia, lymphoma
• Autoimmune or related disorders: SLE, APL syndrome
• Immunodeficiency
• Bone marrow failure and marrow replacement
• Others: hypersplenism
4. QUALITATIVE DISORDERS OF
PLATELET FUNCTION
• INHERITED DISORDERS
Glanzmann thrombasthenia
Bernard Soulier syndrome
Gray platelet syndrome
Dense body deficiency
• ACQUIRED DISORDERS
Medications
c/c renal failure
Cardioulmonary bypass
5. COMMON COAGULATION
DISORDERS
• INHERITED DISORDERS
Hemophilia A and B
von Willebrand disease
Specific factor deficiencies
Factor VII, X, XIII deficiency
Afibrinogenemia
• ACQUIRED DISORDERS
Liver disease
Vit K deficiency
Warfarin overdose
DIC
6. • The process of hemostasis involves platelets, vessel wall and plasma
proteins in a fine balance b/w blood flow and local response to
vascular injury
• Extrinsic pathway is the primary initiating pathway for coagulation
& is measured by PT
• Intrinsic system works as a regulatory amplification loop, measured
by APTT
7.
8. CLINICAL EVALUATION
• Age of onset of bleeding
• Type of bleeding
• Precipitating factors
• Recent onset bleeding – h/o antecedent infection, rash
(Henoch-Schonlein purpura, varicella), icterus (liver failure),
prodromal diarrhoea & associated renal failure(HUS)
• Medications
• Family history – X linked (hemophilia) –only boys are affected,
girls may have bleeding in autosomal dominant (von
willibrand)
• Poor wound healing & prolonged bleeding from umbilical
stump – factor XIII deficiency
12. LABORATORY EVALUATION
• CBC – low platelet count
• Peripheral smear – for abnormal cells or malarial parasites
• LFT, RFT & LDH to rule out hepatitis, occult malignancy, hemolysis &
HUS
• Appropriate evaluation of infections
• Screening tests for DIC if sepsis is suspected
• Bone marrow – increased megakaryocytes
13. MANAGEMENT
• Minimising the risk of h’ge & decreasing the long term side effects
of treatment
• Active bleeding – IV Ig (1g/kg/day) for 1-2days or Anti- D Ig (50-
75mg/kg) in Rh +ve children
• Corticosteroids – Prednisolone (1-4mg/kg/day) for 2-4 weeks and
tapered.
• Severe h’ge – platelet transfusions under cover of steroids
• Chronic ITP – prednisone at low dose on alternate days ,
combination of danazol , vincristine , cyclosporine, azathioprine ,
rituximab , splenectomy , thrombopoetin receptor binding agents.
14. NEONATAL ALLOIMMUNE
THROMBOCYTOPENIA
• Fetal platelets are destroyed by maternal Abs against paternally
inherited Ags on fetal platelets
• H’gic complications including intracranial h’ge may occur within hrs
after birth
• Specific tests are limited – diagnosed by excluding sepsis,
meconium aspiration, IU infections, effect of maternal medications,
maternal SLE
15. • Postnatal Mx – transfusion of washed maternal platelets & close
monitoring until the platelet counts normalize
• Fetus requires serial USG for intracranial h’ge
• IV Ig during pregnancy & at birth along with dexamethasone
16. HEMOPHILIA
• Hemophilia A – factor VIII
• Hemophilia B – factor IX
• C/F of A & B are indistinguishable
• Managed at specialized centres
• Appropriate factor replacement
• Dose of factors required :
VIII = % desired rise in F VIII * bdwt(kg) * 0.5
IX = % desired rise in F IX * bdwt(kg) *1.4
• Judicious physiotherapy to prevent c/c joint d/s, counselling for
injury prevention & monitoring for development of F VIII & IX
inhibitors
17. VITAMIN K DEFICIENCY
• Factors II, VII, IX , X Protein C & S
• Prolonged PT & aPTT
• Vit. K 1 mg s/c at birth to prevent h’gic disease of newborn
• Symptomatic neonates who didn’t receive prophylaxis or have
anticoagulant overdose – vit.K 2-10 mg, repeated till coagulation
studies are normal
• Overt bleeding / suspected liver dysfunction – fresh frozen plasma
18. DIC
SCREENING TESTS
• Peripheral blood film & hemogram – schistocytes &
thrombocytopenia
• PT, aPTT, thrombin time – prolonged
SUPPORTIVE TESTS
• D – dimer – increased
• DIC scoring system based on recommendations of Scientific
Standardization Committee of the International Society on
Thrombosis & Hemostasis
19. Algorithm for diagnosis of DIC using
DIC score
• Risk assessment
? Does patient have an underlying disorder known to be asso. with
DIC? (if yes proceed)
• Order global coagulation tests
Score test results
1)Platelet count Score
• >1,00,000/mm3 0
• 50,000- 1,00,000 1
• <50,000 2
20. 2)Elevated fibrin related marker Score
• no increase 0
• moderate increase 1
• strong increase 2
3)Prothrombin time
• <3s 0
• >3 but <6 1
• >6 2
4)Fibrinogen level
• >1g/L 0
• <1g/L 1
SCORE >/= 5 IS DIAGNOSTIC
21. TREATMENT
1)Hemostatic support(replacement therapy)
• Blood components used in DIC are : fresh frozen plasma,
cryoprecipitate, platelet concentrate, packed red cells
2)Heparin therapy
• Indication – arterial or large vessel venous thrombosis