Bleeding disorders

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					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
Normal Hemostasis
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
Clinical Cases
Who is likely to bleed
•   Obvious
•   Cirrhosis
•   Renal dysfunction
•   Age
•   Drugs
•   Right heart failure
Case 1
• 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
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 36-48
  hours.
• 1-2 mg will decrease INR to therapeutic
• Level within 12-24 hrs
Current practice
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 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 ?
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
• Moderate      1-5% 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 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
Massive bleeding
Questions

				
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