Diagnosis Approach of Bleeding in Children ________________________________ Ketut Ariawati Hematologi Onkologi RSUP Sanglah Denpasar

Introduction Bleeding in a child can be a diagnostic challenge because of the wide range of possible causes Symptoms  such as bruising and epistaxis occur frequently in children without underlying bleeding disorder  So determining which child requires further investigation can be difficult Bleeding disorder can be inherited or acquired Evaluation  comprehensive medical and bleeding history, a complete family history, detailed physical examination and selected laboratory tests

Normal Hemostasis Mechanism by which bleeding from an injured vessel is arrested by formation of a thrombus. Functions To maintain the blood in fluid state To prevent clots in intact vessels To arrest bleeding in injured vessels Components Blood vessels Platelets Plasma coagulation factors Fibrinolytic system

PRIMARY SECONDARY INJURY VESSEL WALL+PLATELET FORMATION OF PLT PLUG STAGES OF HEMOSTASIS INJURY VESSEL WALL+PLATELET FORMATION OF PLT PLUG PRIMARY ACTIVATION OF PLASMA COAGULATION FACTORS FORMATION OF STABLE FIBRIN CLOT SECONDARY DISSOLUTION OF FIBRIN CLOT BY FIBRINOLYSIS

STAGES OF HEMOSTASIS PRIMARY SECONDARY Platelet & vessel wall mediated Occurs within seconds of injury Forms platelet plug Prevent blood loss from capillary,arterioles and venules SECONDARY Coagulation factors mediated Takes several minutes for completion Forms stable fibrin plug Prevents blood loss from large vessels

CAUSES OF BLEEDING 1.Vessel wall disorders 2.Platelet disorders: quantitative or functional. 3.Coagulation factor: deficiency or inhibitors. 4.Combination of these.

VASCULAR DISORDERS ACQUIRED CONGENITAL SENILE PURPURA VASCULAR PURPURA HENOCH SCHONLEIN PURPURA HEREDITARY HEMORRHAGIC TELENGIECTASIA EHLERS DANLOS SYNDROME

PLATELET ABNORMALITIES QUANTITATIVE QUALITATIVE THROMBOCYTOPENIA THROMBOCYTHEMIA THROMBASTHENIA BERNARD-SOULIER SYNDROME DRUGS(ASPIRIN,TXA2, INDOMETHACIN

DISORDERS OF COAGULATION HEREDITARY HEMOPHILIA A(factor VIII deficiency) HEMOPHILIA B(factor IX deficiency) von WILLEBRAND DISEASE DISORDERS OF FIBRINOGEN- HEREDITARY AFIBRINOGENAEMIA HYPOFIBRINOGENAEMIA DYSFIBRINOGENAEMIA ACQUIRED DISSEMINATED INTRAVASCULAR COAGULATION(DIC) LIVER DISEASE VIT K DEFICIENCY MASSIVE TRANSFUSION OF STORED BLOOD ACQUIRED INHIBITORS OF COAGULATION HEPARIN OR ORAL ANTICOAGULANT THERAPY RENAL DISEASE

APPROACH TO A PATIENT OF BLEEDING DIATHESIS CLINICAL EVALUATION : History : Age of first manifestation, Family history of bleeding, Spontaneous or after trauma, Time of manifestation after injury, Ease with which bleeding is controlled, Drug history. Clinical Picture (tabel) 2. LABORATORY APPROACH First line screening tests. Second line specific tests.

Finding Disorders of Coagulation Disorders of Platelets or Vessels Petechiae Rare Characteristic Deep dissecting hematomas Superficial ecchymoses Common; usually large and solitary Characteristic; usually small and multiple Hemarthrosis Delayed bleeding Common Bleeding from superficial cuts and scratches Minimal Persistent often profuse Sex of patient 80–90% of inherited forms occur only in male patients Relatively more common in females Positive family history Rare (exc. vWF , hereditary hemorr. telangiectasia)

Table 118–2. Clinical Manifestations Typically Associated with Specific Hemostatic Disorders Bleeding from stump of umbilical cord and habitual abortions Afibrinogenemia, hypofibrinogenemia, dysfibrinogenemia, factor XIII deficiency Impaired wound healing Factor XIII deficiency Facial purpura in newborns Glanzmann thrombasthenia, severe thrombocytopenia Recurrent severe epistaxis and chronic iron deficiency anemia Hereditary hemorrhagic telangiectasias

Table 118–2. Clinical Manifestations Typically Associated with Specific Hemostatic Disorders Mucocutaneous bleeding Thrombocytopenias, platelet dysfunction, von Willebrand disease Cephalhematomas in newborns, hemarthroses, hematuria, and intramuscular, intracerebral, and retroperitoneal hemorrhages Severe hemophilias A and B, severe deficiencies of factor VII, X, or XIII, severe type 3 von Willebrand disease, afibrinogenemia Injury-related bleeding and mild spontaneous bleeding Mild and moderate hemophilias A and B, severe factor XI deficiency, moderate deficiencies of fibrinogen and factors II, V, VII, or X, combined factors V and VIII deficiency, 2-antiplasmin deficiency

Stepwise approach

ACQUIRED DISORDERS INHERITED DISORDERS Early age of presentation Family history positive More sever Bleeding is the dominant feature Single factor defect Later age of presentation Family history usually negative Less sever Clinical picture is dominated by the underlying disorder e.g.DIC Multiple hemostatic defect

HEMOPHILIA

HEMOPHILIA A group of hereditary genetic disorders that impair the body’s ability to control blood clotting or coagulation Sex-linked X chromosome Females are almost exclusively carriers of the disorder, and may have inherited it from either their mother or father

Causes Hemophilia A X-linked genetic disorder involving a lack of functional clotting Factor VIII and represents 90% of hemophilia cases Hemophilia B X-linked genetic disorder involving a lack of functional clotting Factor IX and more severe but less common Hemophilia C autosomal recessive genetic disorder involving a lack of functional clotting Factor XI

CLINICAL FEATURES: No racial predilection AGE-Bleeding manifestations begin after 6 months of age. SIGNS: Hematomas, Hemarthroses,Hematuria,GI bleeding, Bleeding from laceration , head trauma,spontaneous intra cranial bleeding. Hemorrhage into joints that causes muscle spasm.

Comparison of vWD and Hemophilias Hemophilia A Hemophilia B von Willebrand Disease Factor VIII coagulant activity Low Normal Low or normal von Willebrand factor antigen von Willebrand factor activity Factor IX Ristocetin-induced platelet agglutination Normal, low, or increased at low-dose ristocetin Platelet aggregation Treatment DDAVP* or recombinant VIII Recombinant IX

Inheritance of Hemophilia

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