logging in or signing up Bleeding desorder -shiv sosale Download Post to : URL : Related Presentations : Share Add to Flag Embed Email Send to Blogs and Networks Add to Channel Uploaded from authorPOINT lite Insert YouTube videos in PowerPont slides with aS Desktop Copy embed code: (To copy code, click on the text box) Embed: URL: Thumbnail: WordPress Embed Customize Embed The presentation is successfully added In Your Favorites. Views: 160 Category: Education License: All Rights Reserved Like it (1) Dislike it (0) Added: November 03, 2010 This Presentation is Public Favorites: 0 Presentation Description No description available. Comments Posting comment... By: HDLTharakaWasana (8 month(s) ago) i want to download this ppt Saving..... Post Reply Close Saving..... Edit Comment Close Premium member Presentation Transcript BLEEDING DISORDERS : BLEEDING DISORDERS Email: Shivaprakashsosale@gmail.com +91-7204776479 SAPTHAGIRI INSTITUTE OF MEDICAL SCIENCES(SIMS), BENGALURU . DR.SHIVAPRAKASH SOSALE CHANDRASHEKAR MBBS,MD(Paediatrics) SInCHE …..Reaching unreached Hemostasis-basics : Hemostasis-basics To maintain blood in fluid state Rapid n localised hemostatic plug at the site of vascular # Normal hemostasis depends on delicate balance b/w Blood vessels Platelets Coagulation cascade Inhibitors Fibrinolytic system Classic haemostatic machanism; : Classic haemostatic machanism; Vascular response Platelet adhesion Platelet aggregation Clot formation Clot stabilisation Limiting to site of # by anticoagulents Re-establishment of vasculature by fibriolysis and vascular healing 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 Slide 6: Clotting 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. Platelet dysfunction: : Platelet dysfunction: Inherited Disorders: Bernard-Soulier disease large platelets, failure of adhesion Ab vwf receptor(GPIb complex)on platelet membrane Thrombocytopenia ,gaint plt,BT>20min Glanzmann’s thrombasthenia normal size, failure of aggregation Def .of plt- fibrinogen receptor GPIIb-IIIa Plt size ,count n morphology N,BT inc Acquired Disorders: Drugs - Aspirin, Alcohol, Uremia, Plt-storage granules def: : Plt-storage granules def: Dense body def-absent granules that contain ADP,ATP,Ca2+,serotonin Gray platelet syndrome-absent plt alfa granules Kasabach-marritt synd : Kasabach-marritt synd Gaint hemangioma w localised intravascular coagulation causing thrombocytopenia n hypofibrinogemia Inside hemangioma-plt trapping n activation of coagulation w fibrinogen consuption w generation of FDP A-V malformation within lesion can cause heart failure RX –surgery,lasar,steriod,radiation ,ifn Congenital thrombocytopenic synd; : Congenital thrombocytopenic synd; Cong.amegakaryocytic thrombocytopenia; Only skin n mucous memb –petechie n purpura Bone marrow-ab megakaryocytes Mutaton in stem cell TPO receptor BT curative TAR : TAR Thrombocytopenia B/l radial anamalies Intolerence to cow s milk formula(50%) Eosinophilia n leukemoid reaction AR WAS(wiskott aldrich synd) : WAS(wiskott aldrich synd) Thrombocytopenia w tiny platelets Eczema Rec.infection XR 5% pts develop lymphoreticular malignancies BMT curative OTHERS; : OTHERS; SEBASTIAN SYND EPSTEIN SYND MAY-HEGGLIN SYND FECHTNER SYND Platelet Coagulation : Platelet Coagulation Petechiae, Purpura Hematoma, Joint bl. 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 H/O viral illness 50-60% -EBV,HIV C/F-1to4 yr-sudden onset petechiae n purpura : C/F-1to4 yr-sudden onset petechiae n purpura Splnomegaly rae Spontaneous resolution 70-80%cases present w Ac.ATP <1%pt present w intracranial hemorrhage 20% pts w Ac. ATP go on to have Cr.ATP LAB-Hb, WBC,DC normal In adolescents w new onset ITP ANA to be done to r/o SLE Vascular disorders: : Vascular disorders: Petechiae, purpura, ecchymoses senile purpura vitamin C deficiency (scurvy) Connective tissue disorders Infections – Meningococcus Henoch-Schonlein Purpura-Immu Henoch-Schonlein purpura(anaphylactoid purpura) : Henoch-Schonlein purpura(anaphylactoid purpura) Immune disorder Children Follows infection Small vessel Petechiae with edema and itching. IgA &C3 Henoch-Schonlein purpura : Henoch-Schonlein purpura 15y Male, fever, painful symmetric polyarthritis for a day. During the next two days, edema and palpable purpura developed. Slide 25: Pinkish maculopapular rash-blanch on pressure> palpable purpura Local angioedema Arthritis GIT-diarrhea,pain,ocult blood in stool, Hepatosplenomegaly,LNP Renal-hematuria ,proteinuria,--mesengial deposition of IgA,IgM,C3 & fibrin Difinitive Dsis-BIOpsy TTP; : TTP; PENARD- 1) Fever 2) Microangopthic hemolytic anemia 3) Thrombocytopenia 4) Ab. renal function 5) CNS changes Def of metalloprotein ADAMTS-13 Rx- Plasmaparesis,Steroids,Splenectomy HUS : HUS MC Ac.renal failure in children Triad –1.microangiopathic hemolytic anaemia 2.Trombocytopenia 3.Uremia Etiology—bacterial,viral,drugs, granulomatous condition Pathology—Endothelial cell # C/F---preceded by GI infection Slide 29: Diagnosis—microangiopathic hemolytic anaemia ,thrombocytopenia,ARF PS-Burr cell +,fragmented RBC, Retic inc, Coombs neg XR-Thumb printing colitis RX—plasmaparesis/FFP Thrombocytopenia-TTP : Thrombocytopenia-TTP Slide 31: Von-Willebrand Disease vWF: F-VIII & PLT function. Defective Platelet Adhesion Skin Bleeding Prolonged Bleeding time. Low Factor VIII levels. 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 33: Laboratory evaluation of von Willebrand disease Classification Type 1 Partial quantitative deficiency Type 2 Qualitative deficiency Type 3 Total quantitative deficiency Diagnostic tests: vonWillebrand type Assay 1 2 3 vWF antigen ß Normal ßß vWF activity ß ß ßß Multimer analysis Normal Normal Absent VWD TYPE 1 : VWD TYPE 1 MC ,85% of cases Epistaxis ,bruising and menorrhagia Rx –Desmopressin-0.3microg/kg i.v intranasal(stimate)-150ug/kg <50kg 300ug/kg >50kg VWD TYPE 2A (proteolysis) : VWD TYPE 2A (proteolysis) Abnormal proteolysis of VWF Only smallest VWF present RX-VWF replasement VWF 2B (hyperactive) : VWF 2B (hyperactive) Mutations resulting in “hyperactive” VWF Binds spontaneously to platelets w rapid clearance of both Mod to severe thrombocytopenia+ Rx-infn vwf VWD 2M(binding function) : VWD 2M(binding function) Mutation results in dec platelet binding function of vwf Level of vwf activity dec than vwf antigen VWF 2N (autososmal hemophilia) : VWF 2N (autososmal hemophilia) Dec in factor VIII binding by vwf Binds weakly or not atall to factor VIII Results rapid clearence of factor VIII that weakly complexes to wvf So factor VIII level dec much more than vwf VWD 3 : VWD 3 Undetectable level of vwf Measurable level of f VIII Ddavp no effective Can manifest joint or muscle bleeding simillar to hemophilia PSEUDO (platelet type) VWD : PSEUDO (platelet type) VWD Abnormality of GPIb receptor on platelet In 2B VWD-GPIb receptor on platelet is hyperfunctional n binds plasma vwf spontaeously Slide 41: Acquired desorders Vit k def Liver ds DIC Slide 42: Vitamin K deficiency Source of vitamin K --- Green vegetables Synthesized by intestinal flora Required for synthesis--- Factors II, VII, IX ,X Protein C and S Causes of deficiency ---- Malnutrition Biliary obstruction Malabsorption Antibiotic therapy Treatment----- Vitamin K Fresh frozen plasma 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 Slide 44: NOT AN INTERVAL LIVER DZ : Decreased synthesis of II, VII, IX, X, XI, and fibrinogen Dietary Vitamin K deficiency (Inadequate intake or malabsortion) Dysfibrinogenemia Enhanced fibrinolysis (Decreased alpha-2-antiplasmin) DIC Thrombocytoepnia due to hypersplenism LIVER DZ Slide 46: Common clinical conditions associated withDisseminated Intravascular Coagulation Sepsis Trauma Head injury Fat embolism Malignancy Obstetrical complications Amniotic fluid embolism Abruptio placentae Vascular disorders Reaction to toxin (e.g. snake venom, drugs) Immunologic disorders Severe allergic reaction Transplant rejection DIC : Systemic activation of coagulation Intravascular deposition of fibrin Depletion of platelets and coagulation factors Bleeding Thrombosis of small and midsize vessels with organ failure DIC DIC mechanism; : Coagulation Fibrinolysis Fibrinogen Fibrin Monomers Fibrin Clot (intravascular) Fibrin(ogen) Degradation Products Plasmin Thrombin Plasmin Release of thromboplastic material into circulation Consumption of coagulation factors; presence of FDPs aPTT PT TT Fibrinogen Presence of plasmin FDP Intravascular clot Platelets Schistocytes DIC mechanism; Tests of Hemostasis: : Tests of Hemostasis: Screening tests: Bleeding.T - 10m. Platelet & BV function Prothrombin.T – Extrinsic, aPTT – Instrinsic Thrombin.T – common path. (DIC) Specific tests: Factor assays – hemophilia. Tests of thrombosis – TT, FDP, DDA, Platelet function studies: Adhesion, Aggregation, Release tests. Bone Marrow study 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 51: Blood Coagulation & Tests Coagulation desorders : Coagulation desorders Congenital Hemophilia A(VIII) Hemophilia B(christmas ds) Hemophilia C(XI) Def .of contact factors(non-bleeding desorders) –XII,prekallikrein,HMWK Factor VII def. Factor X def. Factor II def (prothrombin) Fibrinogen def. Factor V def.(parahemophilia) Combined def factor V n VIII Factor XIII def (fibrin stabilising factor) vwd Slide 54: 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 Summary : Summary Disorders of Hemostasis : Disorders of Hemostasis Vascular disorders – Scurvy, easy bruising, Henoch-Schonlein purpura. Platelet disorders Quantitative - Thrombocytopenia Qualitative - Platelet function disorders – Glanzmans Coagulation disorders Congenital - Haemophilia (A, B), Von-Willebrands Acquired - Vitamin-K deficiency, Liver disease Mixed/Consumption: DIC 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 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. SAPTHAGIRI INSTITUTE OF MEDICAL SCIENSES BENGALURU. : SAPTHAGIRI INSTITUTE OF MEDICAL SCIENSES BENGALURU. Thank you SInCHE …..Reaching unreached You do not have the permission to view this presentation. In order to view it, please contact the author of the presentation.
Bleeding desorder -shiv sosale Download Post to : URL : Related Presentations : Share Add to Flag Embed Email Send to Blogs and Networks Add to Channel Uploaded from authorPOINT lite Insert YouTube videos in PowerPont slides with aS Desktop Copy embed code: (To copy code, click on the text box) Embed: URL: Thumbnail: WordPress Embed Customize Embed The presentation is successfully added In Your Favorites. Views: 160 Category: Education License: All Rights Reserved Like it (1) Dislike it (0) Added: November 03, 2010 This Presentation is Public Favorites: 0 Presentation Description No description available. Comments Posting comment... By: HDLTharakaWasana (8 month(s) ago) i want to download this ppt Saving..... Post Reply Close Saving..... Edit Comment Close Premium member Presentation Transcript BLEEDING DISORDERS : BLEEDING DISORDERS Email: Shivaprakashsosale@gmail.com +91-7204776479 SAPTHAGIRI INSTITUTE OF MEDICAL SCIENCES(SIMS), BENGALURU . DR.SHIVAPRAKASH SOSALE CHANDRASHEKAR MBBS,MD(Paediatrics) SInCHE …..Reaching unreached Hemostasis-basics : Hemostasis-basics To maintain blood in fluid state Rapid n localised hemostatic plug at the site of vascular # Normal hemostasis depends on delicate balance b/w Blood vessels Platelets Coagulation cascade Inhibitors Fibrinolytic system Classic haemostatic machanism; : Classic haemostatic machanism; Vascular response Platelet adhesion Platelet aggregation Clot formation Clot stabilisation Limiting to site of # by anticoagulents Re-establishment of vasculature by fibriolysis and vascular healing 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 Slide 6: Clotting 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. Platelet dysfunction: : Platelet dysfunction: Inherited Disorders: Bernard-Soulier disease large platelets, failure of adhesion Ab vwf receptor(GPIb complex)on platelet membrane Thrombocytopenia ,gaint plt,BT>20min Glanzmann’s thrombasthenia normal size, failure of aggregation Def .of plt- fibrinogen receptor GPIIb-IIIa Plt size ,count n morphology N,BT inc Acquired Disorders: Drugs - Aspirin, Alcohol, Uremia, Plt-storage granules def: : Plt-storage granules def: Dense body def-absent granules that contain ADP,ATP,Ca2+,serotonin Gray platelet syndrome-absent plt alfa granules Kasabach-marritt synd : Kasabach-marritt synd Gaint hemangioma w localised intravascular coagulation causing thrombocytopenia n hypofibrinogemia Inside hemangioma-plt trapping n activation of coagulation w fibrinogen consuption w generation of FDP A-V malformation within lesion can cause heart failure RX –surgery,lasar,steriod,radiation ,ifn Congenital thrombocytopenic synd; : Congenital thrombocytopenic synd; Cong.amegakaryocytic thrombocytopenia; Only skin n mucous memb –petechie n purpura Bone marrow-ab megakaryocytes Mutaton in stem cell TPO receptor BT curative TAR : TAR Thrombocytopenia B/l radial anamalies Intolerence to cow s milk formula(50%) Eosinophilia n leukemoid reaction AR WAS(wiskott aldrich synd) : WAS(wiskott aldrich synd) Thrombocytopenia w tiny platelets Eczema Rec.infection XR 5% pts develop lymphoreticular malignancies BMT curative OTHERS; : OTHERS; SEBASTIAN SYND EPSTEIN SYND MAY-HEGGLIN SYND FECHTNER SYND Platelet Coagulation : Platelet Coagulation Petechiae, Purpura Hematoma, Joint bl. 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 H/O viral illness 50-60% -EBV,HIV C/F-1to4 yr-sudden onset petechiae n purpura : C/F-1to4 yr-sudden onset petechiae n purpura Splnomegaly rae Spontaneous resolution 70-80%cases present w Ac.ATP <1%pt present w intracranial hemorrhage 20% pts w Ac. ATP go on to have Cr.ATP LAB-Hb, WBC,DC normal In adolescents w new onset ITP ANA to be done to r/o SLE Vascular disorders: : Vascular disorders: Petechiae, purpura, ecchymoses senile purpura vitamin C deficiency (scurvy) Connective tissue disorders Infections – Meningococcus Henoch-Schonlein Purpura-Immu Henoch-Schonlein purpura(anaphylactoid purpura) : Henoch-Schonlein purpura(anaphylactoid purpura) Immune disorder Children Follows infection Small vessel Petechiae with edema and itching. IgA &C3 Henoch-Schonlein purpura : Henoch-Schonlein purpura 15y Male, fever, painful symmetric polyarthritis for a day. During the next two days, edema and palpable purpura developed. Slide 25: Pinkish maculopapular rash-blanch on pressure> palpable purpura Local angioedema Arthritis GIT-diarrhea,pain,ocult blood in stool, Hepatosplenomegaly,LNP Renal-hematuria ,proteinuria,--mesengial deposition of IgA,IgM,C3 & fibrin Difinitive Dsis-BIOpsy TTP; : TTP; PENARD- 1) Fever 2) Microangopthic hemolytic anemia 3) Thrombocytopenia 4) Ab. renal function 5) CNS changes Def of metalloprotein ADAMTS-13 Rx- Plasmaparesis,Steroids,Splenectomy HUS : HUS MC Ac.renal failure in children Triad –1.microangiopathic hemolytic anaemia 2.Trombocytopenia 3.Uremia Etiology—bacterial,viral,drugs, granulomatous condition Pathology—Endothelial cell # C/F---preceded by GI infection Slide 29: Diagnosis—microangiopathic hemolytic anaemia ,thrombocytopenia,ARF PS-Burr cell +,fragmented RBC, Retic inc, Coombs neg XR-Thumb printing colitis RX—plasmaparesis/FFP Thrombocytopenia-TTP : Thrombocytopenia-TTP Slide 31: Von-Willebrand Disease vWF: F-VIII & PLT function. Defective Platelet Adhesion Skin Bleeding Prolonged Bleeding time. Low Factor VIII levels. 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 33: Laboratory evaluation of von Willebrand disease Classification Type 1 Partial quantitative deficiency Type 2 Qualitative deficiency Type 3 Total quantitative deficiency Diagnostic tests: vonWillebrand type Assay 1 2 3 vWF antigen ß Normal ßß vWF activity ß ß ßß Multimer analysis Normal Normal Absent VWD TYPE 1 : VWD TYPE 1 MC ,85% of cases Epistaxis ,bruising and menorrhagia Rx –Desmopressin-0.3microg/kg i.v intranasal(stimate)-150ug/kg <50kg 300ug/kg >50kg VWD TYPE 2A (proteolysis) : VWD TYPE 2A (proteolysis) Abnormal proteolysis of VWF Only smallest VWF present RX-VWF replasement VWF 2B (hyperactive) : VWF 2B (hyperactive) Mutations resulting in “hyperactive” VWF Binds spontaneously to platelets w rapid clearance of both Mod to severe thrombocytopenia+ Rx-infn vwf VWD 2M(binding function) : VWD 2M(binding function) Mutation results in dec platelet binding function of vwf Level of vwf activity dec than vwf antigen VWF 2N (autososmal hemophilia) : VWF 2N (autososmal hemophilia) Dec in factor VIII binding by vwf Binds weakly or not atall to factor VIII Results rapid clearence of factor VIII that weakly complexes to wvf So factor VIII level dec much more than vwf VWD 3 : VWD 3 Undetectable level of vwf Measurable level of f VIII Ddavp no effective Can manifest joint or muscle bleeding simillar to hemophilia PSEUDO (platelet type) VWD : PSEUDO (platelet type) VWD Abnormality of GPIb receptor on platelet In 2B VWD-GPIb receptor on platelet is hyperfunctional n binds plasma vwf spontaeously Slide 41: Acquired desorders Vit k def Liver ds DIC Slide 42: Vitamin K deficiency Source of vitamin K --- Green vegetables Synthesized by intestinal flora Required for synthesis--- Factors II, VII, IX ,X Protein C and S Causes of deficiency ---- Malnutrition Biliary obstruction Malabsorption Antibiotic therapy Treatment----- Vitamin K Fresh frozen plasma 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 Slide 44: NOT AN INTERVAL LIVER DZ : Decreased synthesis of II, VII, IX, X, XI, and fibrinogen Dietary Vitamin K deficiency (Inadequate intake or malabsortion) Dysfibrinogenemia Enhanced fibrinolysis (Decreased alpha-2-antiplasmin) DIC Thrombocytoepnia due to hypersplenism LIVER DZ Slide 46: Common clinical conditions associated withDisseminated Intravascular Coagulation Sepsis Trauma Head injury Fat embolism Malignancy Obstetrical complications Amniotic fluid embolism Abruptio placentae Vascular disorders Reaction to toxin (e.g. snake venom, drugs) Immunologic disorders Severe allergic reaction Transplant rejection DIC : Systemic activation of coagulation Intravascular deposition of fibrin Depletion of platelets and coagulation factors Bleeding Thrombosis of small and midsize vessels with organ failure DIC DIC mechanism; : Coagulation Fibrinolysis Fibrinogen Fibrin Monomers Fibrin Clot (intravascular) Fibrin(ogen) Degradation Products Plasmin Thrombin Plasmin Release of thromboplastic material into circulation Consumption of coagulation factors; presence of FDPs aPTT PT TT Fibrinogen Presence of plasmin FDP Intravascular clot Platelets Schistocytes DIC mechanism; Tests of Hemostasis: : Tests of Hemostasis: Screening tests: Bleeding.T - 10m. Platelet & BV function Prothrombin.T – Extrinsic, aPTT – Instrinsic Thrombin.T – common path. (DIC) Specific tests: Factor assays – hemophilia. Tests of thrombosis – TT, FDP, DDA, Platelet function studies: Adhesion, Aggregation, Release tests. Bone Marrow study 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 51: Blood Coagulation & Tests Coagulation desorders : Coagulation desorders Congenital Hemophilia A(VIII) Hemophilia B(christmas ds) Hemophilia C(XI) Def .of contact factors(non-bleeding desorders) –XII,prekallikrein,HMWK Factor VII def. Factor X def. Factor II def (prothrombin) Fibrinogen def. Factor V def.(parahemophilia) Combined def factor V n VIII Factor XIII def (fibrin stabilising factor) vwd Slide 54: 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 Summary : Summary Disorders of Hemostasis : Disorders of Hemostasis Vascular disorders – Scurvy, easy bruising, Henoch-Schonlein purpura. Platelet disorders Quantitative - Thrombocytopenia Qualitative - Platelet function disorders – Glanzmans Coagulation disorders Congenital - Haemophilia (A, B), Von-Willebrands Acquired - Vitamin-K deficiency, Liver disease Mixed/Consumption: DIC 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 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. SAPTHAGIRI INSTITUTE OF MEDICAL SCIENSES BENGALURU. : SAPTHAGIRI INSTITUTE OF MEDICAL SCIENSES BENGALURU. Thank you SInCHE …..Reaching unreached