APPROACH TO A BLEEDING CHILD

JISHNU.K.R FINAL MBBS

• Bleeding may occur due to:

1)Defects in platelet a)Thrombocytopenia b)Qualitative disorders of function

2)Coagulation disorders

3)Dysfunctional fibrinolysis

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

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

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

• 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

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

Examination• Ecchymoses, petechia, vascular malformations (hemangioma,

telengiectasia), rashes• Splenomegaly infections, malignancy, collagen vascular dis. or

hypersplenism

ITP / IMMUNE THROMBOCYTOPENIACLINICAL EVALUATION• Antecedent h/o febrile illness• Sudden appearance of bruises & mucosal bleeding, epistaxis, oral

oozing & prolonged bleeds with superficial trauma• Presence of petechie & ecchymoses

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

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.

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

• 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

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

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

DICSCREENING TESTS• Peripheral blood film & hemogram – schistocytes &

thrombocytopenia• PT, aPTT, thrombin time – prolongedSUPPORTIVE TESTS• D – dimer – increased• DIC scoring system based on recommendations of Scientific

Standardization Committee of the International Society on Thrombosis & Hemostasis

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

2)Elevated fibrin related marker Score• no increase 0• moderate increase 1• strong increase 23)Prothrombin time• <3s 0• >3 but <6 1• >6 24)Fibrinogen level• >1g/L 0• <1g/L 1

SCORE >/= 5 IS DIAGNOSTIC

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

THROMBOTIC DISORDERSCLINICAL EVALUATION• Predisposing factors : CHD, recent cardiac catheterization, recent sx,

trauma, use of central venous catheter, nephrotic syndrome, dehydration, sepsis, collagen vascular diseases

• Limb edema, erythema & tenderness on dorsiflexion of foot (+VE HOMAN SIGN) –DVT

• Diminished / absent arterial pulses & cool extremities – arterial thrombosis

• Pulmonary embolism – anxiety, breathlessness, pleuritic chest pain, fever, tachypnoea & cough

• CNS thrombosis – vomiting, lethargy, seizures, weakness• In-utero stroke – seizures & lethargy• Renal vein thrombosis – flank pain & hematuria

LABORATORY EVALUATION• Rule out DIC• Colour doppler – thrombosed vessels• Echocardiography – for venacaval & proximal subclavian vein

thrombosis

• MRI in conjunction with magnetic resonance venography – cerebral venous thrombosis

• CXR & ventilation- perfusion scanning– pulmonary embolism

MANAGEMENT• Unfractionated / LMW Heparin followed by oral warfarin

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