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Bleeding Disorders

Bleeding Disorders. Dr. Farjah H.AlGahtani Assistant Professor of Internal Medicin,Consultant Hematology Director of transfusin Medicin and Blood Bank Department,KSU. Hemostasis. CBC- Plt BT ,(CT) PT PTT. BV Injury. Neural. Damage/contact. Contact. Coagulation Cascade.

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Bleeding Disorders

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  1. Bleeding Disorders Dr. FarjahH.AlGahtani Assistant Professor of Internal Medicin,ConsultantHematology Director of transfusinMedicin and Blood Bank Department,KSU

  2. Hemostasis • CBC-Plt • BT,(CT) • PT • PTT BV Injury Neural Damage/contact. Contact Coagulation Cascade Blood Vessel Constriction Platelet Aggregation Primary hemostatic plug Reduced Blood flow Platelet Activation Fibrin formation Platelet study Antibody tests Factor Assay Stable Hemostatic Plug

  3. HEMOSTASIS • Primary Hemostasis • Blood vessel contraction • Platelet Plug Formation • Secondary Hemostasis • Activation of Clotting Cascade • Deposition & Stabilization of Fibrin • Tertiary Hemostasis • Dissolution of Fibrin Clot • Dependent on Plasminogen Activation

  4. Classification: • Disorders of Blood vessels • Scurvy, senile purpura, Henoch-Schonlein syndrome. • Disorders of Platelets • Thrombocytopenia ITP, TTP, HUS, DIC. • Aspirin therapy, Thrombasthenia, • Disorders of Coagulation • Extrinsic, intrinsic, combined. • Other disorders • Post transfusion purpura, MPS, MDS.

  5. Tests of Hemostasis: • Screeningtests: • Bleeding.T - 10m. Platelet & BV function • Prothrombin.T – Extrinsic, aPTT – Instrinsic • Thrombin.T – common path. (DIC) • Specifictests: • Factor assays – hemophilia. • Tests of thrombosis – TT, FDP, DDA, • Platelet function studies: • Adhesion, Aggregation, Release tests. • Bone Marrow study

  6. Bleeding: Clinical Features • Local - Vs - General, spontaneous . . • Hematoma / Joint Bleeds- Coag • Skin / Mucosal Bleeds – PLT • wound / surgical bleeding – • Immediate - PLT • Delayed - Coagulation

  7. Platelet Coagulation Petechiae, Purpura Hematoma, Joint bl.

  8. Vascular disorders: • Petechiae, purpura, ecchymoses • senile purpura • vitamin C deficiency (scurvy) • Connective tissue disorders • Infections – Meningococcus • Henoch-SchonleinPurpura-Immu

  9. Senile Purpura

  10. Petechiae in Vasculitis (Rocky Mountain Spotted Fever)

  11. Henoch-Schonlein purpura • Immune disorder • Children • Follows infection • Petechiae with edema and itching.

  12. Henoch-Schonlein purpura 20y Male, fever, painful symmetric polyarthritis for a day. During the next two days, edema and palpable purpura developed.

  13. Platelet Disorders - Features: • Mucocutaneous bleeding • Petechiae, Purpura, Ecchymosis. • spontaneous bleeding after trauma • CNS bleeding (severe  plt) • Prolonged bleeding time (BT)

  14. BLEEDING TIME vs. PLATELET COUNT

  15. Idiopathic Thrombocytopenic Purpura (ITP) • Acute - children (post infection) • Chronic - adults ( females, 20-40 yrs) • autoimmune disorder • antiplatelet antibodies (IgG) • IgG coated platelets removed by spleen • Usually  megakaryocytes in BM

  16. Platelet dysfunction: Inherited Disorders: • Bernard-Soulier disease • large platelets, failure of adhesion • Glanzmann’s thrombasthenia • normal size, failure of aggregation Acquired Disorders: • Drugs - Aspirin, Alcohol, Uremia,

  17. Platelet Aggregation Curves

  18. Von-Willebrand Disease: • Coagulation + PLT disorder: • Congenital disorder • Deficiency of vWF molecule • Part of FVIII, • Mediates platelet adhesion • Prolonged Bleeding time • Low Factor VIII & long aPTT • Mucocutaneous bleeding

  19. Von-Willebrand Disease • vWF: F-VIII & PLT function. • Defective Platelet Adhesion • Skin Bleeding • Prolonged Bleeding time. • Low Factor VIII levels.

  20. Coagulation disorders: • Deficiencies of Clotting factors • Onset - delayed after trauma • Deep bleeding • Into joints - Hemarthroses • Into deep tissues – Hematoma • large skin bleed – Ecchymoses

  21. Blood Coagulation & Tests

  22. CT- Large hematoma of psoas muscle

  23. Coagulation Disorders • Laboratory findings: • Normal bleeding time & Platelet count • Prolonged prothrombin time (PT) • deficiencies of II, V, VII, X • Prolonged time (aPTT) • all factors except VII, XIII • Mixing studies - normal plasma corrects PT or aPTT

  24. Factor VIII Deficiency • Classic hemophilia (hemophilia A) • X-linked disorder (affects 1º males) • Most common - severe bleeding • Spontaneous hematomas < 1, 5, 75% • Abnormal aPTT – Intrinsic path. • Diagnosis - factor VIII assay • Treatment - factor VIII concentrate • Cryoprecipitate (less desirable)

  25. Factor IX Deficiency • Christmas disease (Hemophilia B) • X-linked recessive disorder • Indistinguishable from classic hemophilia (F VIII) • Requires evaluation of factor VIII and IX activity levels to diagnose • Treatment - factor IX concentrate • Cryoprecipitate if factor IX unavailable

  26. Secondary Hemostatic Disorders Acquired coagulation disorder: • Vitamin K deficiency - neonates - decreased intestinal flora and dietary intake - oral anticoagulants (coumadin) - fat malabsorption syndromes • Required for factors II, VII, IX, X • Prolonged PT and aPTT

  27. Combined Primary and Secondary Hemostatic Disorders (DIC) Disseminated Intravascular Coagulation • Major pathologic processes - obstetric complications, neoplasms, infection (sepsis), major trauma • Primary - platelet consumption ( bleeding time,  platelets) • Secondary - factor consumption ( PT, aPTT)

  28. Combined Primary and Secondary Hemostatic Disorders Severe Liver Disease • Primary - dysfunctional platelets and/or thrombocytopenia ( BT) • Secondary - decrease in all coagulation factors except vWF ( PT, aPTT) • Vitamin K will promote synthesis of factors II, VII, IX, X

  29. Summary Hemostatic Disorders BT Plt PT PTT Vascular Dis- - - - PLT Disorder - -  - - Factor 8/9 *Congenital - - -  Vit K / Liver *Acquired - -  - Combined (DIC)  -

  30. Summary

  31. CLL-Thrombocytopenia Lymphoid cells Smear cells (Fragile)

  32. Dengue Hemorrhagic fever Platelet deficiency..

  33. Thrombocytopenia-TTP

  34. Summary • Complex system to keep blood fluid • To block leakage on injury. • BV, PLT & Coagulation • Complex inhibitory mechanisms • Complex thrombolysis mechanisms. • Screening tests: BT, CT (PT, aPTT) • Special tests: Factor assay, PLT function etc.

  35. Disorders of Hemostasis • Vasculardisorders – • Scurvy, easy bruising, Henoch-Schonlein purpura. • Plateletdisorders • Quantitative - Thrombocytopenia • Qualitative - Platelet function disorders – Glanzmans • Coagulationdisorders • Congenital - Haemophilia (A, B), Von-Willebrands • Acquired - Vitamin-K deficiency, Liver disease • Mixed/Consumption: DIC

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