BLEEDING DISORDERS. JC Opperman 2012

Transcription

1 BLEEDING DISORDERS JC Opperman 2012

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3 Primary and Secondary Clotting

4 Laboratory Tests Routine screening tests Prothrombin time (PT) (INR) increased in neonates (12-18 sec) Partial thromboplastin time (PTT) increased in neonates (<70 sec) FBC with platelets (D-dimers DIC) Other tests Bleeding time Clotting time Tourniquet test

5 Simplified Clotting Cascade

6 Algorithm for Problems in Primary Clotting

7 Algorithm for problems in Secondary Clotting

8 The problem Exclusion of bleeding tendency in a patient with a strong personal or family history (chronic bleeding disorders) For tonsillectomy Newspaper reports catastrophic outcomes of procedures Dif diagnosis in child abuse The second problem all normal results

9 What we have excluded Low platelets ITP, leukemia, aplastic anemia, etc Abnormal PT and PTT Congenital clotting deficiencies (haemophilia) Vit K deficiencies Severe liver disease Anti-clotting agents

10 Infant with intracranial bleeding Baby referred to our neurologists with extensive intracranial bleeds and severely debilitated First child of young parents Parents caring Routine clotting tests done - normal Von Willebrand tests normal Case reported as child abuse, legal action by authorities and baby in place of safety

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12 Infant with intracranial bleeding Neurology colleague noted that the child s hair looked funny Serum caeruloplasmin and copper done undetectable Diagnosis: Menkes disease associated with abnormal tortuous and fragile cerebral vessels and subdural bleeding

13 Von Willebrand Disease Most common bleeding tendency Quantitative deficiency(80%) Qualitative deficiency (various types) Diagnosis Must specify investigations Specialized laboratory Von Willebrand factor antigen and functional Platelets may be decreased Factor VIII may be decreased Platelet Function Analyzer (PFA-100) Ristocetin platelet aggregation Bleeding time Treatment: Factor VIII Concentrate (derived from plasma); cryoprecipitate

14 PFA-100

15 Congenital Factor XIII deficiency Moderate to severe bleeding tendency Delayed bleeding Delayed separation of umbilical stump, intracranial haemorrhage with little trauma, poor wound healing, miscarriages Stabilizes fibrin and the clot PT, PTT and bleeding time normal Diagnosis: Clot solubility in urea Treatment: Fresh frozen plasma, cryoprecipitate, freeze dried fresh frozen plasma (Bioplasma) Half life 5 7 days. Prophylaxis can be given 2-4 weekly.

16 Platelet Disorders Many medicines cause dysfunctional platelets Inherited platelet disorders The last group often have moderately decreased platelets which are morphologically abnormal Examples: Glanzman thrombasthenia, Bernard Soulier syndrome Diagnosis: Platelet morphology and number Platelet function analyzer Platelet aggregation studies (difficult technique and lots of blood) Bleeding time Platelet transfusion: If it remains high and improve the condition Treatment: Platelet transfusion

17 Platelet Aggregation

18 Disorders of Blood Vessel More important in arterial thrombosis than bleeding tendencies Examples: Ehlers-Danlos syndrome, osteogenesis imperfecta, marfan syndrome Diagnosis: Bleeding time positive or negative Clinical examination

19 Bleeding Time Technically difficult Difficult to standardize Difficult to interpret Low sensitivity

20 Summary Von Willebrand Disease Von Willebrand factor: Antigen and function PFA-100 Ristocetin platelet aggregation studies Family history Bleeding time Plasma derived Factor VIII concentrate Cryoprecipitate Factor XIII deficiency Clot solubility Fresh frozen plasma Cryoprecipitate Platelet disorder Blood vessel disorder Platelet morphology PFA-100 Platelet aggregation Bleeding time ± Bleeding time Clinical examination Platelet transfusion