Presentation on theme: "Bleeding Disorders For Surgeons J. Bormanis Bleeding disorders What are the possibilities What questions have good yield What are screening tests What."— Presentation transcript:
Bleeding Disorders For Surgeons J. Bormanis
Bleeding disorders What are the possibilities What questions have good yield What are screening tests What Lab tests are worrisome and what is the risk
Clinical Approach When did it start ? Dental history Spontanous bruising Bleeding at surgery Bleeding into joints Menstrual bleeding Epistaxis One site only? Where ? When ?
High yield questions Family history Pattern of bleeding - where Difficult to stop or Re-bleeds Drug history Alcohol intake Co Morbid disease
Physical Examination in Bleeding Disorders Check sites of bleeding Is it local or generalized ? what are the manifestations, petichiae, ecchymoses, hematoma ? Are there vessel wall abnormalities, telangectasia, “palpable purpura, perifollicular hemorrhages” ? Are there signs of a connective disease process Are There signs of a systemic disease The type of bleeding should give a good clue as to which part of hemostasis is affected as well as the severity
Laboratory testing History and physical Type of tests guided by clinical features Screening tests Further tests Definitive tests
Screening Tests INR Extrinsic pathway PTT (activated partial thromboplastin time) intrinsic pathway Thrombin time final pathway Platelet count Bleeding time – PFA (not useful)
Laboratory tests further testing INR PTT TT thrombin time Factor assays Tests of fibrinolysis platelet count Bleeding time platelet function tests Special tests
Inerpretation of tests If isolated abnormality likely a single defect eg PTT - possible hemophilia, vWd If unexplained do mixing test for inhibitor IF more than one abnormality then more complex eg. INR and PTT - vitamin K- Coumadin eg. PTT,TT heparin eg INR, PTT, TT, Platelets DIC or liver disease
Case 1 It is Friday at 4:40pm Lab calls Your patient is being preped for urgent surgery. INR 6.5 What to do ?
Why INR’s go out of control
Vitamin K Warfarin affects factors II,VII,IX and X These are the vitamin K dependent factors Can reverse warfarin effect Takes time Available forms ?
Efficacy of route of administration
Reversing INR wityh vitamin K Depends on clinical scenario Complete reversal Partial reversal (too high INR) IV or oral forms prefered For complete reversal 5-10 mg IV q12h for 2 doses will reverse completely in hours. 1-2 mg will decrease INR to therapeutic Level within hrs
Current practice Sent as 2 vials in a 50 cc mini bag to infuse at 3c/min
Case 2 You are on call for ENT and are asked to see an 18 year old girl with refractory nosebleed. The nose is packed and bleeding does not stop. You notice a few bruises Blood sent off to lab. The lab calls at 6:00 Pm with a “critical” platelet count of 10 What is likely diagnosis What to do ?
ITP Immune thromboctopenic purpura What is needed for diagnosis Bone marrow examination Anti platelet antibodies When isolated and very low ITP is most likely diagnosis Could be a part of another disease but not likely (SLE, inf mono) Does it require hospitalization ?
ITP continued If mucosal bleeding platelets are less than 6 Needs action Steroids IVIG Anti D What about splenectomy New treatments Rituximab TPO agonists
Case3 A 48 year old woman appears in emerg with jaundice of 3 weeks duration Exam – jaundice - some RUQ pain an palpation Blood tests CBC Hgb 125, WBC 7.6 Plat 345 INR 2.6 ptt 42 What is likely diagnosis What to Do ?
Vitamin K deficiency Obstructive jaundice Malabsorption of Vit K dependent factors Older people at risk Post surgery at risk Treatment Oral or IV Vitamin K
Case 4 A 54 year old male comes to emerg feeling unwell. Exam Mild jaundice, some telangectasis on skin Mod ascites. CBC - Hgb 110 WBC 2.5 plat 68 INR 1.6 Ptt 41 TT 25 What is likely diagnosis ?
Hepatic dysfunction - Cirrhosis Liver makes and degrades Coagulation is affected by decreased production and impaired degradation of activated factors Chronic DIC Splenomegaly Trearment only if bleeding Liver transplant
Case 5 18 year old male scheduled for tonsillectomy History of easy bleeding Exam normal no bruises CBC normal INR 1.1 PTT 45 What is likely diagnosis ? How to diagnose ?
Hemophilia X linked bleeding disorders characterized by spontaneous development of large hematomes in deep tissues. May lead to joint bleeding, or into other closed structures Joint cavity bleeding leads to deformed joints bleeding may be spontaneous or asssociated with mild or moderate injury
Hemophilia types Hemophilia A absent or decreased factor VIII Hemophilia B lack of factor IX similar in symptoms to Hemophilia A Hemophilia A is 10 times more common than hemophilia B
Genetics of Factor VIII Single chain polypeptide Produced mainly in Liver remember linked to VWf Gene deletion - no factor VIII Point mutation - abnormal factor VIII Base deletion - Abnormal Factor VIII Coded on X chromosome -therefore only males affected (transmitted by female carriers)
Hemophilia types Subclassified by level of factors Levels correspond to clinical symptoms Mild 5-30% factor activity Moderate1-5% activity Severe<1% activity
Hemophilia - Clinical Picture Mild- do not develop spontaneous bleeding, but do bleed after injury or surgery Many patients have sever disease Joint Bleeding results in severe disability hemarthroses chronic arthritis muscle bleeds Social, economic,psychological problems
Case 6 17 year old girl with mennorhagia History of easy bruising Possible history of easy bruising CBC normal INR 1.1 PTT 32 (2 sec prolonged) What is diagnosis How to diagnose ? Treatment ?
Von Willebrand’s Disease Most frequent inherited bleeding disorder 1% - 1/100 of western population less severe than hemophilia Disease results from a decrease or absence of Von Willebrand factor for platelet adhesion Affects primary hemostasis
Von Willebrand’s Disease and Factor VIII VW factor produced in megakaryocytes and endothelial cells Coded on chromosome 12 Autosomal dominant inheritance Large molecule, and multimeric Monomers undergoglycolisation and multimerization before secretion Different multimer size = disease
Von Willebrand’s Disease and Factor VW is carrier for factor VIII Factor VIII-VWf complex Factor VIII protein carried in circulation as complex with VWf Reacts with platelet via GP Ib Therefore can be problems with platelets and factor VIII
Clinical features of Von Willebrand’s Disease Generally mild bleeding - often unrecognized until surgery or injury epistaxis, menorrhagia, easy bruising, dental and post operative bleeding Can be severe in certain types Requires accurate diagnosis Requires specific treatment
VW -types Type I most frequent, quantitave defect (decreased VWf ) Type II qualitative defect (abnormal VWf ) Type III severe, rare, (absence of VWf )
How to diagnose Von Willebrands disease Clinical history Factor VIII level Bleeding time Measure VWf and perform aggregation tests Do gel electrophoresis for multimers
Anti platelet agents ASA Not likely to create problems Safer to give if there for cardiovascular reasons Clopidogrel If elective stop before. Minimum 3 days More than 5 days likely unnecessary
What are the critical issues ?
Massive Transfusion Protocol The Ottawa Hospital Ottawa, Ontario