1. Approach to the Bleeding Child

2. Evaluation  History Current Bleeding Medical Family  Physical exam  Selected laboratory investigations

3. Current History  an increase in severity or frequency of bleeding from one site e.g.; nose.  bleeding from unusual sites such as joints or internal organs.  excessive bleeding for the degree of the trauma experienced.

4. Bleeding History  Easy bleeding/bruising  into soft tissues/joints (think clotting factor deficiency, coagulation disorder)  epistaxis, menorrhagia, gum bleeding, mucous membranes (suggestive of platelet disorder, vascular)  GI bleeding, hematuria  previous surgeries, immunizations, tooth extractions, circumcision, etc  excessive bleeding with fractures, minor cuts  Significant or not? Generalized or localized?. Acquired or hereditary?  Time of presentation.

5. Bleeding history  Vascular and platelet dysfunction : Usually spontaneous subcutaneous or mucus membrane bleeding eg; purpura, petechiae, epistaxis  Coagulation factor deficiency: Bleeding usually occurs deep into joints, muscles, retroperitoneal space; post traumatic bleeds are often prolonged (sometimes hours).

6. Medical History  Age, Sex  Recent illness, medications?  Presence of chronic disease e.g.; renal or liver disease, malabsorption?  Nutritional status: decreased hepatic synthesis  Screen for other cytopenias systemic symptoms (wt loss, night sweats, fever, weakness) recurrent infections, sepsis anemia (fatigue, pallor)  Remember non-accidental trauma! Consider child abuse with unusual bruising

7. Family History  Detailed family history of bleeding disorders? sex linked recessive (hemophilia A,B,WAS) autosomal recessives disease : clotting factors defeciency 2,5,7,10,11,13. autosomal dominant : (VWF, qualitative platelet disorders, dysfibronogenimia) autoimmune disorders  Consanguinity  Race/descent e.g. factor XI in Ashkenazi Jews

8. Physical Examination Vital signs: Pulse, BP, RR, Temp General appearance, pallor Mucosal bleeding, joint/muscle hematomas Petechiae/purpura/ecchymosis, Splenomegaly +/- hepatomegaly Lymphadenopathy Signs of sepsis

9. Ecchymoses, Purpura, Petechiae

10. Physical examination Sites of hemorrhage – abdominal, pelvis, long bones, etc Evidence of chronic disease, malignancy. Delayed wound healing? Factor 13 deficiency, dysfibrinogenemia Musculoskeletal examination for bleeding and extensibility, dysmorphic features

11. Labs: Screening tests  Complete blood count  Peripheral blood film  PT/INR (extrinsic and common pathways)  Tissue factor, II, V, VII, X, fibrinogen  aPTT (intrinsic and common pathways) -II, V, VIII, IX, X, XI, XII, Fibrinogen  Bleeding time

12. TestMechanism testedRef value (Varies)Disorder PTExtrinsic & common pathways <12s beyond neonate, 12-18s in neonate Vit K related bleeding, HDN, malabsorption, Liver disease, oral anticoagulants aPTTIntrinsic & common pathways 20-40s beyond neonate, 70s in neonate Hemophilia, VWD, heparin, DIC, lupus anticoagulant, Factor XI & XII deficiency Thrombin Time Fibrinogen to fibrin10-15s beyond neonate, 12-17s in neonate Fibrin split products, DIC, heparin, uremia, hypofibrinogenemia Bleeding Time Hemostasis, capillary & platelet function 3-7minPlatelet dysfunction, thrombocytopenia, aspirin, von Willebrand disease Platelet count Platelet number150,000-450,000/ mm 3 Causes of Thrombocytopenia Thin FilmPlatelet number, size, RBC morphology Large platets-peripheral destruction, Fragmented RBCs- microangiopathic process

13. PT and aPTT Prolonged APTT Defect in Intrinsic No change in PT No change in APTT Defect in Extrinsic Prolonged PT Prolonged APTTDefect in common Prolonged PT

14. Additional labs  Specific factor assays  Mixing study (patient plasma 1:1 normal plasma)  Thrombin Time and Fibrinogen measurement  Urea clot lysis test (Factor XIII def)  Platelet function testing  VWF antigen and activity testing  Genetic testing