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Coagulation Disorders

This detailed guide explores various coagulation disorders through case studies and laboratory investigations. Highlights include a 4-year-old boy presenting with sudden bruising and petechiae, laboratory results showing thrombocytopenia, and treatment options such as IVIg and steroids detailing potential outcomes. Further analysis covers infections, disseminated intravascular coagulation (DIC), immune and congenital thrombocytopenias, and hemophilia, emphasizing the importance of timely diagnosis and effective management. This resource aims to enhance understanding of complex coagulation issues and their implications on patient care.

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Coagulation Disorders

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  1. Coagulation Disorders Corrina Mc Mahon

  2. Laboratory investigations • PT: VII, X, V • APTT; XII, XI, IX, VIII • TT; Fibrinogen • D dimers; fibrin breakdown

  3. Case 1 • 4 yr old boy • URTI 2 weeks ago • Sudden onset bruising/petechiae • PH: Nil • FH: Nil • Physical examination:

  4. Investigations • FBC: Hb 11g/dl; WCC 8x10^/l; Platelets <10x10^9/l • PT 14 sec ; APTT 33 sec; Fibrinogen 2.0g/l • Treatment options: Nil; IVIg; Steroids • Outcome: 90% recovery; 10% chronic

  5. Congenital Thrombocytopenia • Dysfunctional platelets • Bernard Soulier • Grey platelet syndrome • Wiskott-Aldrich syndrome • Normal Platelet function • May-Hegglin • TAR

  6. Newborn infant Intracranial Haemorrhage No dysmorphic features 1st child No liver/spleen palpable FBC Hb 18.5g/dl WCC 10 x x 109/l /l Platelets 10 x 109/l /l Coagulation screen PT 15 sec. (13-16) APTT 41 sec (28-36) Case 2

  7. Differential diagnosis • Infection • DIC • Immune Thrombocytopenia • Alloimmune • Isoimmune • Congenital Thrombocytopenia • TAR syndrome • Wiscott Aldrich Syndrome • Von Willebrands disease • Type 2B • A-V malformations

  8. Alloimmune Thrombocytopenia • Incidence 1:1000-5000 births • IgG antibodies • HPA1a 80% • HPA5b 15% • 50% occur in 1st pregnancy • Bleeding can be in utero or after birth Treatment • Platelets • IVIg • ?Steroids

  9. Isoimmune Thrombocytopenia • Maternal anti-platelet IgG • Placental Passage • Thrombocytopenia nadir ~5days post-partum • History & examination of mother • Treatment • IvIg ± steroids

  10. Disseminated Intravascular Coagulopathy • Infection • Symptoms and Signs • Petechiae • Bruising • Bleeding • Laboratory results • Anaemia • Thrombocytopenia • ↑PT/ ↑APTT/↓Fibrinogen/ ↑d dimers

  11. Haemophila • Inherited Bleeding Disorder • Factor VIII/FIX deficiency • X-Linked Inheritance • Carrier XX may have low levels • Spontaneous mutation

  12. Inheritance of Haemophilia

  13. Life Expectancy In Haemophilia

  14. Bleeding problems in Haemophilia Factor LevelType of Bleed <1% Spontaneous/severe 2%-5% Mild trauma/ occasionally spontaneous >5% Trauma/Surgery

  15. Intracranial Bleeds • At Birth • Injury • Admission • Factor Concentrate • Scanning • Observation • Neurosurgery

  16. Forearm Bleed

  17. Joint bleedSynovial inflammation and hyperaemiaSynovial overgrowth and Bone resorptionFurther BleedJoint Destruction

  18. Joint Bleeding

  19. Chronic Joint Bleeding

  20. The role of prophylaxis in the prevention of joint injury Lofqvist, Nilsson et al ( Journal Int. Medicine May 1997): 34 patients aged 7-22yrs. Age at commencement of prophylaxis - 1-4.5yrs. 79% had no joint problems and the rest had no deterioration in joint abnormalities. Liesner,Khair, Hann, ( BJH Mar 1996) 27 children aged 1.3-15.9yrs. No. of bleeds/yr pre-prophylaxis-14.5 and post - 1.5. 20 children had evidence of arthropathy which improved on prophylaxis.

  21. Prophylaxis The Irish Data (1992-1997) Bleeds/yr, pre-prophylaxis, 9.5-106 (mean 38) Bleeds/yr, post-prophylaxis, 0-9 (mean 3.5) Development of inhibitors, 2 - low level (<1Bu) and transient (< 1 year)

  22. Factor VIII T½ = 8 hours Frequency – three times/week Dose – 20-40iu/kg Factor IX T½ = 18 hours Frequency – twice/week Dose – 50iu/kg Prophylaxis

  23. Dose Adjustment • Growth • Break through bleeds

  24. Management of Acute Bleeds • Rest • Factor Concentrate • FVIII; 35-50iu/kg • FIX; 70-100% (7-10iu/ml) • Wt x desired rise x 1.25 • Continuous infusion • FVIII • 50iu/kg bolus; infusion 4iu/kg/hr • FIX • 100% bolus; infusion 6-8iu/kg/hr

  25. Mild Factor VIII Deficiency • Factor VIII • DDAVP • 0.3mcg/kg/30 min • Antifibrinolytic therapy

  26. Haemophilia The problems • Bleeding • Destructive arthropathy • Addiction • Infection • Inhibitors

  27. Inhibitors • Anti-FVIII Antibodies - IgG • Incidence: 10-20% • High responding or lowlevel/transient •  Familial incidence (x6) • Majority <10yrs • Occur within first 25 treatment days •  Bleeding

  28. Management of Inhibitors • Acute Bleeding episodes • FVIIa • Immune Tolerance • High Dose 200-300iu/kg/d x 1-3 yrs • Cyclophosphamide/FVIII/IVIg • 50iu/kg/d x 1->12m • 25iu/kg/d x 1->12m

  29. Autosomal Inheritance Abnormal VWF S/S: easy bruising, mucosal bleeds, heavy periods Treatment: antifibrinolytic agents DDAVP Plasma derived factor (Fanhdi) Lab Investigations FVIIIc VWF:Ag VWF:RCF Bleeding time VWF Multimers Von Willebrands Disease

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