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Practical Approach to a Bleeding Child - Peri Kamalakar_ M.D

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					Practical approach to a bleeding
              child

           Peri Kamalakar,MD
                 Director
   The Valerie Fund Children’s Centers
      For Cancer &Blood Disorders
                    At
   Saint Barnabas Health Care System
  Associate Director, Hemophilia Center
    Newark Beth israel Medical Center
Practical approach to a bleeding child

OBJECTIVES:
• Overview of hemostasis

• Clinical approach in making a diagnosis

• Review the most common bleeding conditions

• Discuss the current treatment strategies
Overview of Haemostasis




                               INJURY
                                                                    Tissue Factor
                         Collagen Exposure
   VASOCONSTRICTION


                      Platelet Adhesion and release
                                 reaction

      Serotonin                              Platelet Phospolipid    Coagulation


                           Thromboxane A2
                                ADP


                         Platelet aggregation                          Thrombin


                      Primary haemostatic plug
                                                                        Fibrin


                       Stable haemostatic plug
                                                                                    Fibrinolysis
Overview of Haemostasis




                               INJURY
                                                                    Tissue Factor
                         Collagen Exposure
   VASOCONSTRICTION


                      Platelet Adhesion and release
                                 reaction

      Serotonin                              Platelet Phospolipid    Coagulation


                           Thromboxane A2
                                ADP


                         Platelet aggregation                          Thrombin


                      Primary haemostatic plug
                                                                        Fibrin


                       Stable haemostatic plug
                                                                                    Fibrinolysis
                            Coagulation cascade
Intrinsic Pathway                                        Kinins                  HMW Kininogen
                                                                  Kallikrein             Contact Activation

                                                         XII                             Prekallikrein
                                                                                 XIIa
                                                                  XIa                    XI

Extrinsic Pathway   VII                     VIIa         IXa      Ca++           IX
                                     Ca++                         VIIIa                  VIII
                                Tissue Factor                      Ca++
                                                                  Phospholipid
Common Pathway             Xa                                                  X
                                          Va                                   V
                                          Ca++

                                          Phospholipid                         XIII
                           II                        IIa

                                                                               XIIa
                    Fibrinogen                                    Fibrin       XIII
PRACTICAL APPROACH TO A CHILD WITH
BLEEDING HISTORY

• HISTORY – HISTORY – HISTORY

 >AGE OF ONSET
   > SEX
      >FREQUENCY
        >LOCATION / TYPE OF BLEEDING
          >DURATION OF BLEEDING
            > MEDICATIONS
             > ASSOCIATED SYMPTOMS
              > REVIEW OF SYSTEMS
Approach to a bleeding patient
 –What is the type of bleeding disorder?

  Primary hemostasis – Vascular causes
                          Platelets-Number vs.
                                    Function
  Fibrin formation – clotting factors

  Premature clot dissolution- post clot
  formation
. Approach to a bleeding patient
 –Is a bleeding tendency present?

 Easy Bruising
 Mucosal bleeding
 Menorrhagia
 Surgical Hemorrhage – Procedure vs.Diathesis
 Postpartum Hemorrhage
 Joint and Muscle bleed –Severity of trauama
. Approach to a bleeding patient
 –Is the disorder Familial or Acquired?


  Family history – MOTHER & OTHER
 FEMALE MEMBERS IN THE IMMEDIATE
  FAMILY – Detailed Menstrual history
• Vascular causes –
          First and foremost rule out
 infectious causes – “Meningococcemia”

Vasculitis – Henoch-Schonlein Purpura

Hemangiomas- Kassalback-Merritt syndrome
•   Petechiae and Purpura
•   ���� Infectious
•   – Meningococcemia
•   – Rocky mountain spotted
•   fever
•   – Group A strep
•   – Atypical measles
•   – Echovirus 9, 4, 7
•   – Epstein-Barr virus
•   – Coxsackie virus A9
•   ���� Non-infectious
•   – Normal platelets
•   ���� HSP
•   ���� Coagulation disorders
•   ���� Trauma
•   – Low platelets
•   ���� ITP
•   ���� Leukemia
PRACTICAL APPROACH TO A CHILD WITH
BLEEDING HISTORY

• PHYSICAL EXAMINATION-
            > PETICHEAE
               >ECHYMOSES
               >JOINT BLEED &DEEPSEATED HEMATOMAS
               > HEPATOSPLENOMEGALY
               >SIGNIFICANT LYMPHADENOPATHY
               > ACTIVE AND PLAYFUL VS. ILL LOOKING
               > DYSMORPHIC FEATURES
               > CAFÉ-AU-LAIT SPOTS
               >TELANGIECATIC VESSELS
               >HEMANGIOMAS
PRACTICAL APPROACH TO A CHILD WITH
BLEEDING HISTORY
• LABORATORY WORK UP-
P.M.D -
          > C.B.C./PLATELET COUNT
           >PERIPHERAL SMEAR- MORPHOLOGY
           > P.T. [Prothrombin time]
           > a.P.T.T. [ Activated partial thromboplastin time]
      -----------------------------------------------------------------
   Hemophilia service --
      > T.T. [Thrombin time]
           > Bleeding time
           >Platelet aggregation studies
           > Factor assay
Pandora’s box: coagulation
test
            The results are as good as the
              sample is.

Standards: Time from sample to test: PT 24 hours ,PTT 4
  hours.

 Blood/citrate ratio: 9 :1.
Bleeding disorders
• Platelets–
           Acquired causes much more common

           Thrombocytopenia more common than
            functional defects

   Inherited disorders – both number &functional defects
    are extremely rare
PLATELETS – NUMBER
    Acquired causes are most common
    I.T.P.
    Infections

CONGENITAL THROMBOCYTOPENIAS
   T.A.R. syndrome
• I.T.P.-
      Most have benign &limited course
Treatment options-
      Conservative –wait &watch
Aggressive-
      Steroids
      IvIGG
      Rhogam
      Rituximab
              PLATELETS

• Functional disorders-
         Acquired- Aspirin; Uremia

   Inherited –
            Glanzman’s
            Bernard-Soulier
            Gray platelet syndrome
von Willebrand Disease

• The most common inherited bleeding
 disorder

• Occurs in 1% of the population

• Less than 10% of patients have bleeding
 events due to vWD
Inheritance of Type 1 vWD
Functions of vWF
• Binds to platelet receptor GP Ib and to
    subendothelial structures such as collagen
    serving as bridge between platelets and
    subendothelium in damaged vessels
•   Acts as bridge between adjacent platelets in
    vessels with high shear (arterioles) forming
    small platelet aggregates
•   Binds to circulating factor VIII protecting it and
    prolonging FVIII t1/2 in the circulation from 2 to
    8-12 hours
Symptoms of vWD

• Easy bruisability
• Epistaxis or gingival bleeding
• Menorrhagia
• Post-partum hemorrhage
• Post-surgical bleeding
• Bleeding post-dental extraction
Sub types of VW

Classification of vWD of vWF
Type 1   Partial quantitative deficiency

Type 2           Qualitative variants of vWF

•            A                   Absence of HMW vWF multimers

•            B                 Same as 2A and increased affinity
                               for platelet gp Ib
•        M                       Abnormal function not caused by
                                absence of HMW multimers

•    N                  Decreased affinity for factor VIII

Type 3           Complete deficiencey of vWF & Behave as Severe
                 Hemophilia A
Treatment Guidelines in VWD
     TYPE        TREATMENT
        1           DDAVP
      2A        DDAVP/FVIII-VWF
      2B            FVIII-VWF
      2M            FVIII-VWF
      2N            FVIII-VWF
       3            FVIII-VWF
DDAVP (1-desamino-8-D-arginine
vasopressin)
• Parenteral form: DDAVP (for IV
  or SC use, 0.3 ug/kg)
• Highly concentrated intranasal
  spray form: Stimate nasal spray
  (150-300 ug )
Hemophilia
Hemophilia
• Caused by an absence or decreased
  amount of a procoagulant –
• VIII -Hemophilia A affects ~ 1:5000
  males
• XI -Hemophilia B affects ~ 1:30000
  males
• XI –Hemophilia C – Rare /Ethnicity
Epidemiology

  Incidence:     Hemophilia A - 1:5,000
                 Hempohilia B – 1: 30, 000


               Hemophilia A

                                             Other


                                    Hemophilia B
Inheritance
Inheritance
Woman can have hemophilia

  • Lyonization of the normal X
    chromosome
  • Turner syndrome ( XO)

  • Father with hemophilia/ mom as a
    carrier
  • vW type 2 N ( Normandy)
HEMOPHILIA SEVERITY LEVELS
• Severe <1% activity level - Spontaneous bleeds



• Moderate 1 to 5% activity --Trauma/surgery bleeds
                              Occasional joint bleeds




• Mild 5 to 30% activity -   Major trauma/surgery
                             Rare joint bleeds
Factor replacement
• 1 u/kg raises FVIII levels 2%
  1/2 life : 12 hrs

• 1 u/kg raises FIX levels 1 %
  1/2 life 20-24 hrs
Minor Bleeding Episodes
• Early joint bleeds
• Soft tissue & muscle bleeds
• Nose & gum bleeding not responding
  to local measures
• Treatment of minor bleeding
  episodes
  – 40 - 50% correction
  – FVIII : 25 units / kg
  – FIX : 50 units / kg
Major Bleeding Episodes
 • Advanced soft tissue & muscle bleeds
 • Head & neck injuries
 • Gastrointestinal bleeding
 • Advanced joint bleeding
 • Treatment of major bleeding episodes
   • 80 – 100 % correction
   • FVIII : 50 units / kg
   • FIX : 100 units / kg
Current Products
• Plasma Products: plasma-derived factor VIII concentrate
• Porcine Factor:
    – Use was halted due to parvovirus/retrovirus sequences
      discovered
• Recombinant products:
     – First Generation: Recombinate, Kogenate, Helixate
     – Second Generation: Kogenate FS, Helixate FS
     – Third Generation: Advate
•   DDAVP:
     – Causes release of factor VIII/vWF
     – Increased factor activity in 30-60”
     – For mild hemophiliacs and mild bleeding symptoms
Replacement therapy: Joint
disease
• Prophylaxis
  –Primary
  –Secondary


• Intensive infusion therapy

• Dose escalation modified prophylaxis
Clinical Severity
Chronic Joint
Hemophilia Treatment Center Team
Members
• Patient / Family     • Primary Care
• Hematologist         • Infectious Disease
• Nurse                • Genetics
• Social Worker        • Pharmacy
• Physical Therapist   • Dental
• Orthopedist          • Hepatologist
Basis for Comprehensive
Care
• Hematologist
     – Assumes overall care
•   Musculoskeletal
     – Orthopedic Surgeon, Physical therapist
•   Nursing
     – Coordination of home/clinic care for rapid treatment
       at the earliest symptoms suggestive of a bleed
•   Dental
•   Genetic Counseling
•   Infectious Disease
•   Psychosocial
     – social worker
Role of Hemophilia Treatment
Centers
• State-of-the-art medical treatment for
  persons with hemophilia through out the
  life span
• Education
• Research
• Outreach
• Model of comprehensive care for
  chronic disease
The Past…
Present




…the promise of achieving your potential
Made possible by a STRONG & Dedicated
 Hemophilia parent association and
 dedicated NJHA staff
Thank you

				
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posted:8/22/2012
language:English
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