Presentation on theme: "J. Bormanis/ cg edits When did it start ? Dental history Spontanous bruising Bleeding at surgery Bleeding into joints Menstrual bleeding."— Presentation transcript:
J. Bormanis/ cg edits
When did it start ? Dental history Spontanous bruising Bleeding at surgery Bleeding into joints Menstrual bleeding Epistaxis One site only? Where ? When ?
Family history Pattern of bleeding - where Difficult to stop or Re-bleeds Drug history Alcohol intake Co Morbid disease
History and physical Type of tests guided by clinical features Screening tests Further tests Definitive tests
Blood Coagulation & Tests Thrombin time
Platelet count INR Extrinsic pathway PTT (activated partial thromboplastin time) intrinsic pathway Thrombin time final pathway fibrinogen
Factor assays Tests of fibrinolysis platelet function tests Special tests
Cirrhosis Renal dysfunction Age Drugs Right heart failure
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 (Cirrhosis) New anticoagulants Dabigatran: TT, PTT Rivaroxaban: antiXa, PT/INR
INRPTTTTPLATDx > > >60396
It is Friday at 4:40pm Lab calls You are patient is being preped for urgent surgery. INR 6.5 What to do ?
Why INR’s go out of control
Warfarin affects factors II,VII,IX and X These are the vitamin K dependent factors Can reverse warfarin effect (Vitamin K, prothrombin complex concentrates)
Efficacy of route of administration
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
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 ?
What is needed for diagnosis? 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 ?
If mucosal bleeding platelets are less than 10 Needs action Steroids IVIG Anti D splenectomy Newer treatments Rituximab TPO agonists
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 ?
Obstructive jaundice Malabsorption of Vit K dependent factors Older people at risk Post surgery at risk Treatment Oral or IV Vitamin K
A 54 year old male comes to office 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 ?
Liver makes and degrades Coagulation is affected by decreased production and impaired degradation of activated factors Chronic DIC Splenomegaly Treatment only if bleeding Liver transplant
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 ?
X linked bleeding disorders characterized by spontaneous development of large hematomas 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 associated with mild or moderate injury
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
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)
Subclassified by level of factors Levels correspond to clinical symptoms Mild 5-30% factor activity Moderate1-5% activity Severe<1% activity
Mild- do not develop spontaneous bleeding, but do bleed after injury or surgery Many patients have severe disease Joint Bleeding results in severe disability hemarthroses chronic arthritis muscle bleeds Social, economic,psychological problems
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 ?
Most frequent inherited bleeding disorder 1% of western population less severe than hemophilia Disease results from a decrease or absence of Von Willebrand factor for platelet adhesion Affects primary hemostasis
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
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
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
Type I most frequent, quantitave defect ( decreased VWf ) Type II qualitative defect ( abnormal VWf ) Type III severe, rare, ( absence of VWf )
Clinical history Factor VIII level Antigen and activity tests (Ristocetin cofactor activity) Do gel electrophoresis for multimers
ASA Not likelely to create problems Safer to give if there for cardivascular reasons Clopidogrel If elective stop before. Minimum 3 days More than 5 days unnecessary
common DVT, PE Splanchnic/portal vein thrombosis Signs and Symptoms: Pain, swelling, erythema in an extremity SOB, CP, tachycardia, hypoxia Diagnosis Clinical prediction models: Wells’ score D-dimer testing Imaging: US, CTPA, VQ Underlying risk factors Thrombophilia: inherited, acquired
Treatment Low molecular weight heparin warfarin* IV unfractionated heparin warfarin HIT (T): to consider if drop in platelet count in patient on a heparin agent *continue LMWH for first 6 months after diagnosis of cancer associated VTE New oral anticoagulant agents Rivaroxaban (Xarelto) Apixaban (Eliquis)