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Bleeding and Coagulation Disorders PowerPoint Presentation

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Description : Bleeding and Coagulation disorders.

Tags : Hemophilia, Coagulation disorders , Bleeding, Dr Nishant Verma

Published on : Oct 04, 2020 | Views : 4594 | Downloads : 31

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Slide 1-

Case 6yr boy is brought to the OPD with complaints of recurrent painful swelling of the Lt Knee joint since 2yr of age. He also has a history of prolonged bleeding from cut sites. One maternal uncle of the child died due to prolonged bleeding following a minor surgery. O/E, No petechiae/purpura. Lt Knee joint swollen, tender
Slide 2-

Hemophilia and Coagulation Disorders Dr Nishant Verma
Slide 3-

Hemostatic Mechanism Platelet adhesion Platelet aggregation Clot formation Clot stabilization Limitation of clotting to the site of injury by regulatory anticoagulants, and Re-establishment of vascular patency through fibrinolysis and vascular healing
Slide 4-

von Willebrand‘s Factor (vWF) binds to subendothelial collagen. Conformational changes occur in vWF allow it to bind to the GP Ib/IX receptor on platelets, causing adhesion BERNARD- SOULIER SYNDROME
Slide 5-

The GP IIb/IIIa receptor complex changes conformation allowing binding of fibrinogen. Fibrinogen acts as a glue binding platelets together. GLANZMANN’s Thrombasthenia
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Clotting Factors
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Waterfall cascade: Prothrombin Fibrinogen Factor X Common Pathway Fibrin Thrombin Factor Xa
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HEMOPHILIA
Slide 9-

Overview of fibrinolytic mechanism
Slide 10-

von Willebrand Factor
Slide 11-

Classification of bleeding disorders Primary Hemostatic defect Platelet disorder Congenital Acquired Von Willebrand Disease Coagulation Disorders (Clotting factor deficiency) Acquired Inherited Vascular
Slide 12-

Classification of bleeding disorders Source: Nathan and Oski’s Hematology of Infancy and Childhood. 7th Edition, Pg 1450
Slide 13-

Clinical Approach to bleeding disorders History Physical Examination Laboratory Evaluation
Slide 14-

Clinical Approach to bleeding disorders History Nature of bleeding- - Immediate vs delayed - Superficial vs deep - Surgical / dental history Family H/O bleeding- - Others involved ? - Males only? (x-linked) - Consecutive generations Medication history- -NSAID, Heparin (patients with central lines) Others- Liver / renal disease
Slide 15-

Clinical Approach to bleeding disorders Physical Examination Bruises- - Number - Location - Site Petechiae Joint bleeding Other Physical findings- - Jaundice - Skeletal deformity - Hepatosplenomegaly
Slide 16-

Clinical Approach to bleeding disorders Screening Laboratory Evaluation Platelet count / morphology Coagulation profile Prothrombin time (PT) Activated partial thromboplastin time (APTT) Bleeding time (BT)
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BT / CT Bleeding time 3-9 min Clotting time 3-6 min
Slide 18-

Coagulation profile Sample collection Citrated tube Gently mixed Immediate transport
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Coagulation profile PT Method Normal – 10-11s INR = (Patient PT/Control PT)ISI Isolated  PT APTT Method Normal – 26-35s Isolated  APTT  PT +  APTT TT TT
Slide 20-

Advanced tests Factor assays Testing for vWD Platelet function analyzer (PFA 100)
Slide 21-

Classification of bleeding disorders Primary Hemostatic defect Platelet disorder Congenital Acquired Von Willebrand Disease Coagulation Disorders (Clotting factor deficiency) Acquired Inherited Vascular
Slide 22-

Coagulation Disorders: Acquired Vitamin K deficiency Liver disease Accelerated destruction of coagulation factors Inhibitors of coagulation Miscellaneous
Slide 23-

Vitamin K Dependent Proteins Dietary Vitamin K Vitamin K Reductase
Slide 24-

Vitamin K deficiency Liver Disease or Vit. K deficiency Hemorrhagic disease of the newborn Biliary obstruction (neonatal cholestatic disorders) Malabsorption of vitamin K (celiac disease, ulcerative colitis) Drugs Vit.K antagonists – warfarin, phenytoin Broad-spectrum antibiotics – alter gut flora
Slide 25-

Manifestations Diagnosis: PT, APTT Management Vitamin K oral / sc / iv Repeat PT after 6hr Prevention Prophylactic Vit K to at risk population Vitamin K deficiency
Slide 26-

Coagulation Disorders: Inherited Hemophilia A Hemophilia B Factor XIII Deficiency Prothrombin Deficiency Factor V Deficiency Factor VII Deficiency Factor X Deficiency Factor XI Deficiency Factor XII Deficiency Prekillikrein Deficiency High Molecular Weight Kininogen Deficiency a2-antiplasmin Deficiency Plasminogen Activator Inhibitor Deficiency Incidence 1 in 5000 to 10,000 Rare
Slide 27-

27 Often called ‘the disease of kings’ because it was carried by many members of Europe’s royal family. Queen Victoria of England was a carrier of Hemophilia HEMOPHILIA
Slide 28-

X-linked recessive Hemophilia A (FVIII def): 80-85% Hemophilia B (FIX def) Mutations of the clotting factor gene Family H/O bleeding common, - generally affects males on the maternal side - 1/3 no family history – due to new mutations
Slide 29-

TYPES 29
Slide 30-

30 Severity of Hemophilia is defined by measured level of clotting factor activity
Slide 31-

HEMOPHILIA
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32 Bleeding can happen anywhere in the body. Following an injury / surgery or rarely spontaneous. CLINICAL MANIFESTATIONS
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33 CLINICAL MANIFESTATIONS Musculoskeletal bleeding Deep bleeding into joints and muscles Begin when child reaches toddler age. In toddlers ankle the most common site. Later knees and elbow become common sites.
Slide 34-

34 Target joint Repeated bleeds Hemophilic arthropathy
Slide 35-

35 Other manifestations Intracranial haemorrhage Hematuria Traumatic bleeding Venipuncture
Slide 36-

Hemophilia : Diagnosis Screening tests Normal PT , Raised APTT. Mixing studies Definitive diagnosis specific factor VIII or IX by assays F VIII deficient plasma F IX deficient plasma
Slide 37-

37 Carrier state and Genetic testing Three approaches: Patient and family history Coagulation-based assays: unreliable DNA testing: GOLD standard Prenatal diagnosis
Slide 38-

Case 6yr boy is brought to the OPD with complaints of recurrent painful swelling of the Lt Knee joint since 2yr of age. He also has a history of prolonged bleeding from cut sites. O/E, Lt Knee joint swollen, tender Investigations ??? PT- Normal, APTT – 90sec (Control – 25sec) Mixing study: Corrected with factor IX deficient plasma Factor VIII assay: < 1% activity Went to a dentist for tooth extraction. Developed uncontrolled bleeding following the procedure. How will you manage ?
Slide 39-

Hemophilia: Management Issues to be considered Lifestyle modifications Available therapeutic options Inhibitors complicating Hemophilia Prophylactic factor therapy Transfusion transmitted infections
Slide 40-

Hemophilia: Management Lifestyle modifications: Goal - Prevention of bleeding. - Avoid drugs that affect platelet function -NSAIDs - paracetamol - safe for analgesia. - Regular exercise to promote strong muscles, protect joints, and improve fitness. - Avoid contact sports ; swimming and cycling encouraged. - Recognize early signs of bleeding - a tingling sensation or “aura”. - trained to seek treatment at this stage. - Carry identification indicating the diagnosis, severity, and contact information .
Slide 41-

Hemophilia: Management Available pharmacological agents Factor concentrates Cryoprecipitate Fresh Frozen Plasma and Cryo-Poor Plasma Adjuvant Pharmacological Options Desmopressin (DDAVP) Tranexamic acid Epsilon aminocaproic acid (EACA)
Slide 42-

Hemophilia: Management Factor concentrates
Slide 43-

WHF Recommendations for target factor levels
Slide 44-

Hemophilia: Management Cryoprecipitate - Prepared by slow thawing of FFP at 4°C for 10–24 hours. - Contains – FVIII, vWF, fibrinogen, & FXIII (not FIX or XI). - supernatant - cryo-poor plasma and contains other coagulation factors VII, IX, X, and XI. - FVIII /bag of cryoppt is 60-100 units (avg-80 units) in a 30-40 ml vol. -does not contain factor IX, so no use in Haemophilia B Concerns : - factor content of individual packs variable. - not subjected to viral inactivation procedures
Slide 45-

Hemophilia: Management Fresh Frozen Plasma FFP can be used to treat both hemophilia A &B 1 U FFP contains about 160-250ml plasma with activity of ~80%. Rate and total dose limited by the risk of acute or chronic circulatory overload. How to use Thaw. Transfuse over how many minutes. Reusing after thawing Disadvantages: No viral inactivation F level >20-25% difficult to achieve
Slide 46-

Hemophilia: Management Desmopressin Only effective in mild hemophilia A - single i.v. infusion of 0.3 mg/kg expected to boost FVIII level 3-6 fold Ineffective in severe hemophilia A No value in hemophilia B - does not affect FIX levels Nasal spray available - useful for home treatment of minor bleeding problems.
Slide 47-

Hemophilia: Management Tranexamic acid / EACA Antifibrinolytic agent, competitively inhibits activation of plasminogen to plasmin. Valuable in controlling bleeding from mucosal surfaces (e.g., oral bleeding, epistaxis, menorrhagia) - dental surgery - eruption of teeth Tranexa dose for children - 25 mg/kg up to three times daily - 500 mg tablet can be crushed, dissolved in water for topical use on bleeding mucosal lesions.
Slide 48-

48 Management of Hemophilic arthropathy Analgesics, ice packs ( 5 minutes on, 10 minutes off, for as long as the joint feels hot), avoidance of weight bearing and immobilisation. Factor replacement- most important Physiotherapy
Slide 49-

Hemophilia: Management Inhibitors: Suspected - when no / inadequate response to factor replacement. Detected by: Measuring factor levels after factor replacement Mixing studies Treatment: low-responders - specific factor at a much higher dose High responders - alternative agents like bypassing agents : as recombinant factor VIIa and prothrombin complex concentrates.
Slide 50-

Hemophilia: Management Prophylactic Therapy Administration of clotting factors at regular intervals to prevent bleeding - Patients with clotting factor level > 1% seldom have spontaneous bleeding 25-40 IU/kg of clotting factor concentrates - 3 times/week for hemophilia A - twice a week for hemophilia B Expensive but preservation of joint function & improved QOL Administered by subcutaneous access port of central line
Slide 51-

51 Comprehensive care Comprehensive team including hemophilia specialist, nurse coordinator, social worker, psychologist, physiotherapist, orthopaedic surgeon, primary care physician, financial counsellor and sometimes infectious disease specialist Provided primarily through comprehensive hemophilia treatment centres
Slide 52-

Case 6yr boy is brought to the OPD with complaints of recurrent painful swelling of the Lt Knee joint since 2yr of age. He also has a history of prolonged bleeding from cut sites. O/E, Lt Knee joint swollen, tender Investigations ??? PT- Normal, APTT – 90sec (Control – 25sec) Mixing study: Corrected with factor IX deficient plasma Factor VIII assay: < 1% activity Went to a dentist for tooth extraction. Developed uncontrolled bleeding following the procedure. How will you manage ?
Slide 53-

Classification of bleeding disorders Primary Hemostatic defect Platelet disorder Qualitative Quantitative Von Willebrand Disease Coagulation Disorders (Clotting factor deficiency) Acquired Inherited Vascular
Slide 54-

Classification of bleeding disorders Primary Hemostatic defect Platelet disorder Qualitative Quantitative BERNARD- SOULIER SYNDROME GLANZMANN’s Thrombasthenia Diagnosis BT Platelet counts Failure to agglutinate by Ristocetin PFA Flowcytometry Genetic testing
Slide 55-

Classification of bleeding disorders Primary Hemostatic defect Platelet disorder Qualitative Quantitative (Thrombocytopenia) Impaired production Aplastic anemia Leukemia MDS B12/Folate deficiency Hereditary (TAR) Increased destruction ITP SLE Thrombotic microangiopathy (HUS) Sequestration Hyperspenism SPURIOUS THROMBOCYTOPENIA
Slide 56-

Case 2 yr girl is brought to the ER with complaints of red colored spots over entire body for last 3 days. H/O, URI 2wk back. O/E, Afebrile. No Pallor Spleen : just palpable DDx ? Investigations ? Platelet count: 12000/mm3
Slide 57-

Pathogenesis of ITP
Slide 58-

Definitions Newly diagnosed ITP: diagnosis to 3 months Persistent ITP: 3 to 12 months from diagnosis Chronic ITP: lasting for more than 12 months Immune Thrombocytopenia (ITP) Platelet count less than 100 × 109/L in absence of other causes or disorders that may be associated with thrombocytopenia Source: The American Society of Hematology 2011 evidence-based practice guideline for immune thrombocytopenia
Slide 59-

Investigations Complete blood count Peripheral smear Bone marrow aspiration ???
Slide 60-

Treatment of Newly diagnosed ITP General Tt Education Activity limitation No NSAIDs Careful follow up Observation only Vs Pharmacological Tt
Slide 61-

Case 2 yr girl is brought to the ER with complaints of red colored spots over entire body for last 3 days. H/O, URI 2wk back. O/E, Afebrile. No Pallor Spleen : just palpable Observation only Daily follow up advised. Comes next day with Epistaxis and gum bleeding. What next???
Slide 62-

Treatment of Newly diagnosed ITP General Tt Education Activity limitation No NSAIDs Careful follow up Observation only 1st line Pharmacological Tt Corticosteroids IVIG Anti D Vs
Slide 63-

1st line Medical Therapies in ITP IVIG Y Y Anti D RBC Anti D
Slide 64-

1st line Medical Therapies in ITP Corticosteroids
Slide 65-

2nd line Treatment options Rituximab High dose Dexamethasone Other immunosuppressants Splenectomy
Slide 66-

Classification of bleeding disorders Primary Hemostatic defect Platelet disorder Qualitative Quantitative von Willebrand Disease Coagulation Disorders (Clotting factor deficiency) Acquired Inherited Vascular
Slide 67-

von Willebrand Disease Pathophysiology Types Manifestations Treatment
Slide 68-

DIC Causes Manifestations Diagnosis Treatment Activation of coagulation cascade
Slide 69-

Thank You