bleeding disorders

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Bleeding Disorders& Clotting disorders : 

Bleeding Disorders& Clotting disorders

Hemostasis : 

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

HEMOSTASIS : 

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

Classification: : 

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.

Tests of Hemostasis: : 

Tests of Hemostasis: Screening tests: Bleeding.T - 10m. Platelet & BV function Prothrombin.T – Extrinsic, aPTT – Instrinsic Specific tests: Factor assays – hemophilia. Platelet function studies: Adhesion, Aggregation, Release tests. Bone Marrow study

Bleeding: Clinical Features : 

Bleeding: Clinical Features Hematoma / Joint Bleeds- Coag dis Skin / Mucosal Bleeds – Bleeding dis

Platelet Coagulation : 

Platelet Coagulation Petechiae, Purpura Hematoma, Joint bl.

Vascular disorders: : 

Vascular disorders: Petechiae, purpura, ecchymoses senile purpura vitamin C deficiency (scurvy) Connective tissue disorders Infections – Meningococcus Henoch-Schonlein Purpura-Immu

Slide 9: 

Senile Purpura

Slide 10: 

Petechiae in Vasculitis (Rocky Mountain Spotted Fever)

Henoch-Schonlein purpura : 

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

Henoch-Schonlein purpura : 

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

Platelet Disorders - Features: : 

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

Idiopathic Thrombocytopenic Purpura (ITP) : 

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

Platelet dysfunction: : 

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,

Von-Willebrand Disease: : 

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

Slide 18: 

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

Coagulation disorders: : 

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

Slide 20: 

Blood Coagulation & Tests

Slide 21: 

CT- Large hematoma of psoas muscle

Coagulation Disorders : 

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

Factor VIII Deficiency : 

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)

Factor IX Deficiency : 

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

Secondary Hemostatic Disorders : 

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

Combined Primary and Secondary Hemostatic Disorders (DIC) : 

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)

Combined Primary and Secondary Hemostatic Disorders : 

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

Summary Hemostatic Disorders : 

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

Summary : 

Summary

Dengue Hemorrhagic fever : 

Dengue Hemorrhagic fever Platelet deficiency..

Summary : 

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.

Slide 32: 

thank you & Happy new year

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