By-Dr.Anupam
:
1. Overview of hemostasis
2. Clinical approach in making a diagnosis
3. LAB diagnosis
4. Review the most common bleeding
conditions
Active process that clots blood in areas of blood
vessel injury yet simultaneously limits the clot size
only to the areas of injury.
HEMOSTASIS
Components:
1. Vessel wall
2. Platelets
3. Coagulation proteins
4. Anticoagulant proteins
5. Fibrinolytics
Overview of Haemostasis
INJURY
Collagen Exposure
Platelet Adhesion and release
reaction
Platelet aggregation
VASOCONSTRICTION
Serotonin Platelet Phospolipid
Thromboxane A2
ADP
Primary haemostatic plug
Stable haemostatic plug
Tissue Factor
Coagulation
Thrombin
Fibrin
Fibrinolysis
CLOTTING FACTORS
Protein C/S
Factor 8 & 5
Antithrombin
3
PRACTICAL APPROACH TO A CHILD
WITH BLEEDING HISTORY
HISTORY
A.onset of symptoms
B.Sites of bleeding
C.Perinatal history
D.Gynecologic bleeding
E.Medication
F. Diet
Family history
HISTORY
A)Onset-
• age
• acute vs chronic
• Timing of bleeding-immediate vs delayed
1. Mucocutaneous
a. Epistaxis
• Duration, frequency,
• Associated trauma (nose picking, allergy,infection)
b. Oral (bleeding after tooth brushing, after dental
extractions requiring sutures/packing)
c. Bruising (number, sites, size, raised [other than
extremities],
d. Gastrointestinal bleeding
SITES OF BLEEDING
2. Deep
a. Musculoskeletal
• Hemarthroses, unexplained arthropathy
• Intramuscular hematomas
b. Central nervous system hemorrhage
c. Genitourinary tract
3. Surgical
a. Minor (sutures, lacerations, poor or delayed wound
healing)
b. Major
• Tonsillectomy and adenoidectomy
• Abdominal surgery
Contd……
C. Perinatal history
a. Superficial (bruising, petechiae)
b. Deep
• Circumcision
• Central nervous system bleeding
• Gastrointestinal bleeding
• Cephalohematoma
• Unexplained anemia or hyperbilirubinemia
• Delayed cord separation, bleeding after cord
separation
c. Vitamin K administration
d. Maternal drugs
D.MEDICATIONS
a. Aspirin and NSAIDS
b. Anticoagulants
c. Antibiotics
d. Anticonvulsants
E. Diet
a. Vitamin K
b. Vitamin C
II. Family History
.
PHYSICAL EXAMINATION-
• Healthy and sick looking
• Vitals signs and growth parameters
• Joint examination for :
A)chronic arthropathy
B)joint laxity
• Extremities examination fot thumb or radial
anomalies(TAR)
• Lymphadenopathy/hepatosplenomegaly
Contd………
Skin examination-
• pallor,
• hematoma,
• petechiae,
• ecchymosis,
• telangiectasias,
• poor wound healing,lax skin
Hemarthrosis
PETECHIAEECCHYMOSIS,
Approach to a bleeding patient
LABORATORY WORK UP-
1) C.B.C./PLATELET COUNT
2) PERIPHERAL SMEAR- MORPHOLOGY
3) P.T. [Prothrombin time]
4) a.P.T.T. [ Activated partial thromboplastin
time]
Control bleeding; give fluids arrange blood;order
CBC,PT,aPTT stat
Normal PT,aPTT and decreased platelet
Normal morphology and size of platelets
Perform bone marrow examination
Normal megakaryocytes Decreased megakaryocytes
Peripheral platelet destruction
1. ITP
2. Alloimmune thrombocytopenia
3. Drug induced throbocytopenia
4. TTP
5. HELLP
6. Splenic sequestration
7. Others(sepsis,malignancy)
Decreased production
1. Heriditary thrombocytopenia
2. Fanconi anemia
3. Myelopthisic disorders
4. Drug induced aplasia
5. Viral aplasia
6. Acquired megakaryocytic purpura
Control bleeding; give fluids arrange blood;order
CBC,PT,aPTT stat
Prolonged PT,aPTT and decreased
platelet
Prolonged PT and /or aPTT
Normal platelets
Sick child Healthy child
Investigate for liver disease
1. Acute viral hepatitis
2. Drug induced hepatitis
3. Autoimmune hepatitis
4. Hepatotoxic agent
ingestion
Both PT and
aPTT prolonged
PT normal and
PTT prolonged
VIT K def
• Give vit k
• probiotics
Clotting factor
def
Hemophilia
A
B
C
Control bleeding; give fluids arrange blood;order
CBC,PT,aPTT stat
Healthy child Sick child
Bleeding due to
local factors like
trauma,
anatomic
abnormalities
Qualitative platelet defects
1. Order platelet
aggregation studies
2. Order platelet surface
GP analysis
Compromised vascular
integrity
1. Sepsis
2. Hypoxia
3. Acidosis
4. prematurity
Normal PT and APTT and normal platelet counts
PT PTT FIBRINO-
GEN
PLATELETS
FDPs Clinical
keys
DIC ↑ ↑ ↓ ↓ ↑ Shock
Liver failure
↑ ↑ ↓ N/↓ N Jaundice
Vitamin K
def ↑ ↑ N N N Malabsorpt
ion
Sepsis
without
shock
↑ ↑ N N ↑/N Fever
DD’s for DIC
Some common bleeding conditions
Causes
a) Infectious-CMV,HCV,HIV
b) Vaccination
c) SLE
d) APLA synd
e) Drugs-quinidine,sulfonamide,heparin
f) CVID
g) Lymphoproliferative disorder
 Newly diagnosed ITP-diagnosis to 3months
 Persistent ITP-3to 12months of diagnosis
 Chronic ITP->12months
 Corticosteroid dependence ITP-need for
ongoing or repeated administration of steroid
to maintain platelet count in excess of 30x109
or avoid bleeding.
 Severe ITP-bleeding at presentation of
sufficient magnitude requiring treatment or
occurrence of new bleeding symptoms
requiring intervention
 RefractoryITP-presence of severe ITP occuring
after splenectomy
MANAGEMENT
GRADE 1 MINOR BLEEDING
FEW PETECHIAE(<100)
<5 SMALL MUCOSAL BLEED
Observation
GRADE 2 MILD BLEEDING
>100 PETECHIAE
>5LARGE BRUISE(>3cm)
NO MUCOSAL BLEEDS
Observation
Treatment in selected
childred
GRADE 3 MODERATE BLEEDING
OVERT MUCOSAL BLEED
MENORRHAGIA
Intervention to reach
grade1 or gr2 in selected
children
GRADE 4 MUCOSAL BLEEDS OR
SUSPECTED INTERNAL
BLEEDS
INTERVENTION
DRUGS APPROX
RESPONSE
RESPONSE
TIME
TOXICITY
Iv anti D
50-75mcg/kg
50-77% Within
24hrs
Headache,fever
IVIG
0.8-1g/kg SD
>80% 1-2days Fever
Prednisolone
1-2mg/kg-14d
or
4mg/kg 4days
¾ patient
respond
depending on
dose
2-7days Gastritis
Wait &
watch
2/3 pt
improve
spontaneously
within
6months
Days to
month
-
a)Children and adolscents
 PLTs <50,000/dl and bleeding
 PLTs<50,000/dl and invasive procedure
 PLTs <20,000/dl and marrow failure and
hemorragic factors
 PLTs any counts,but with PLTs dysfunction
plus bleeding or on an invasive procedure
Infants within the first 4 months of life:
 PLTs <1lakh/dl and bleeding
 PLTs<50,000/and an invasive procedure
 PLTs<1lakh/dl and clinically unstable
 PLTs any counts,but with PLTs
dysfunction plus bleeding or on an
invasive procedure
Caused by an absence or decreased amount of a
procoagulant –
1. VIII -Hemophilia A affects ~ 1:5000 males
2. IX -Hemophilia B affects ~ 1:30000 males
3. XI –Hemophilia C – Rare /Ethnicity
• Hallmark –HEMARTHROSIS
• Easy bruising ,intramuscular
hematomas,heamarthrosis
• Life threatening condition like bleed into
iliopsoas muscle,GI bleed
 Prolonged PTT(reduced level of 8 & 9)
 Other screening test of hemostatic
mechanism(platelet,bleeding time,PT)are
normal.
 Specific assay for factors VIII & IX confirms
the diagnosis
 Dose calculation of r factor 8 and factor 9
 Dose of factor 8
=%desired(rise in f8)xbody weight(kg)x0.5
 Dose of factor 9
=%desired(rise in f9)xbody weight(kg)x1.4
 Factor 8 concentrates-
A)Plasma -derived factor VIII as well as factor VIII/von
Willebrand factor (vWf) concentrates are prepared from
cyroprecipitates and contain vWf and moderately
enriched factorVIII.
B) Recombinant factor -concentrates are prepared in
animal cell cultures using biotechnological procedures
Package Sizes-250/500/1000/1,500/2,000 U/package.
storage temperature for concentrates is 2–8 °C
Diluent-sterile water
Lyophilised powder
Route-IV
baxject
-DDAVP’s
 VWF tethers platelet to injured endothelium
 VWF serves as carrier protein for factor 8
 Stored in WEIBEL PALADE BODIES in
endothelial cells
 Facilitates its ability to bind to platelets by
GP1b receptor.
 Easy bruisability
 Epistaxis or gingival bleeding
 Menorrhagia
 Post-surgical bleeding
 Bleeding post-dental extraction
Sub types of VW
Type 1 Partial quantitative deficiency of vWF
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
N - Decreased affinity for factor VIII
Type 3 Complete deficiencey of vWF & Behave as Severe
Hemophilia A
 VWf concentrates-
 Desmopressin sprays
 antifibrinolytics
VWD treatment
factor XIII is responsible for the crosslinking of
fibrin to stabilize the fibrin clot,
symptoms -delayed hemorrhage
Typically, patients have trauma 1 day and then
have a bruise or hematoma the next day.
C/f
 mild bruising, delayed separation of the
umbilical stump beyond 4 wk in neonates,
 poor wound healing,
screening tests –for hemostasis are normal
 The normal clot remains insoluble in the
presence of 5M urea, whereas in a patient with
XIII deficiency, the clot dissolves.
 The half-life of factor XIII is 5-7 days
 There is a heat-treated, lyophilized
concentrate of coagulation factor XIII
available to treat bleeding episodes or for
prophylaxis.
Blood products
• Indication
1)to increase oxygen delivery in an anemic patient
2)acute blood loss greater than 25-40% in blood volume.
Dose-10ml/kg of RBC will increase Hb by 2.5-3g/dl
Recommneded temp-33-35 C
 Can be single or pooled whole blood
 1U/10 kg=increase upto 50,000
 Stored in mechanical agitator at 22 C upto
5days
 Freezing plasma at -18 C within 8hrs of
separation from whole blood
 Contains all coagulation factors
 Stored at -18C for 1yr
 Prior to administration—thawed at 36-38C
 10-15ml/kg over 30-120mins
 Contains fibrinogen(150mg/bag),
factor 8(80u/bag),VWf,factor 13
 Highly concentrated form of plasma protein
that settle down at bottom as ppte when frozen
plasma is slowly thawed at 1-6 C
 Dose-10-20 ml/kg
Thank you
Anuupam ppt for bleeding child
Anuupam ppt for bleeding child

Anuupam ppt for bleeding child

  • 1.
  • 2.
    : 1. Overview ofhemostasis 2. Clinical approach in making a diagnosis 3. LAB diagnosis 4. Review the most common bleeding conditions
  • 3.
    Active process thatclots blood in areas of blood vessel injury yet simultaneously limits the clot size only to the areas of injury. HEMOSTASIS Components: 1. Vessel wall 2. Platelets 3. Coagulation proteins 4. Anticoagulant proteins 5. Fibrinolytics
  • 4.
    Overview of Haemostasis INJURY CollagenExposure Platelet Adhesion and release reaction Platelet aggregation VASOCONSTRICTION Serotonin Platelet Phospolipid Thromboxane A2 ADP Primary haemostatic plug Stable haemostatic plug Tissue Factor Coagulation Thrombin Fibrin Fibrinolysis
  • 5.
  • 6.
    Protein C/S Factor 8& 5 Antithrombin 3
  • 7.
    PRACTICAL APPROACH TOA CHILD WITH BLEEDING HISTORY HISTORY A.onset of symptoms B.Sites of bleeding C.Perinatal history D.Gynecologic bleeding E.Medication F. Diet Family history
  • 8.
    HISTORY A)Onset- • age • acutevs chronic • Timing of bleeding-immediate vs delayed
  • 9.
    1. Mucocutaneous a. Epistaxis •Duration, frequency, • Associated trauma (nose picking, allergy,infection) b. Oral (bleeding after tooth brushing, after dental extractions requiring sutures/packing) c. Bruising (number, sites, size, raised [other than extremities], d. Gastrointestinal bleeding SITES OF BLEEDING
  • 10.
    2. Deep a. Musculoskeletal •Hemarthroses, unexplained arthropathy • Intramuscular hematomas b. Central nervous system hemorrhage c. Genitourinary tract 3. Surgical a. Minor (sutures, lacerations, poor or delayed wound healing) b. Major • Tonsillectomy and adenoidectomy • Abdominal surgery Contd……
  • 11.
    C. Perinatal history a.Superficial (bruising, petechiae) b. Deep • Circumcision • Central nervous system bleeding • Gastrointestinal bleeding • Cephalohematoma • Unexplained anemia or hyperbilirubinemia • Delayed cord separation, bleeding after cord separation c. Vitamin K administration d. Maternal drugs
  • 12.
    D.MEDICATIONS a. Aspirin andNSAIDS b. Anticoagulants c. Antibiotics d. Anticonvulsants E. Diet a. Vitamin K b. Vitamin C II. Family History .
  • 13.
    PHYSICAL EXAMINATION- • Healthyand sick looking • Vitals signs and growth parameters • Joint examination for : A)chronic arthropathy B)joint laxity • Extremities examination fot thumb or radial anomalies(TAR) • Lymphadenopathy/hepatosplenomegaly
  • 14.
    Contd……… Skin examination- • pallor, •hematoma, • petechiae, • ecchymosis, • telangiectasias, • poor wound healing,lax skin Hemarthrosis PETECHIAEECCHYMOSIS,
  • 15.
    Approach to ableeding patient LABORATORY WORK UP- 1) C.B.C./PLATELET COUNT 2) PERIPHERAL SMEAR- MORPHOLOGY 3) P.T. [Prothrombin time] 4) a.P.T.T. [ Activated partial thromboplastin time]
  • 16.
    Control bleeding; givefluids arrange blood;order CBC,PT,aPTT stat Normal PT,aPTT and decreased platelet Normal morphology and size of platelets Perform bone marrow examination Normal megakaryocytes Decreased megakaryocytes Peripheral platelet destruction 1. ITP 2. Alloimmune thrombocytopenia 3. Drug induced throbocytopenia 4. TTP 5. HELLP 6. Splenic sequestration 7. Others(sepsis,malignancy) Decreased production 1. Heriditary thrombocytopenia 2. Fanconi anemia 3. Myelopthisic disorders 4. Drug induced aplasia 5. Viral aplasia 6. Acquired megakaryocytic purpura
  • 17.
    Control bleeding; givefluids arrange blood;order CBC,PT,aPTT stat Prolonged PT,aPTT and decreased platelet Prolonged PT and /or aPTT Normal platelets Sick child Healthy child Investigate for liver disease 1. Acute viral hepatitis 2. Drug induced hepatitis 3. Autoimmune hepatitis 4. Hepatotoxic agent ingestion Both PT and aPTT prolonged PT normal and PTT prolonged VIT K def • Give vit k • probiotics Clotting factor def Hemophilia A B C
  • 18.
    Control bleeding; givefluids arrange blood;order CBC,PT,aPTT stat Healthy child Sick child Bleeding due to local factors like trauma, anatomic abnormalities Qualitative platelet defects 1. Order platelet aggregation studies 2. Order platelet surface GP analysis Compromised vascular integrity 1. Sepsis 2. Hypoxia 3. Acidosis 4. prematurity Normal PT and APTT and normal platelet counts
  • 19.
    PT PTT FIBRINO- GEN PLATELETS FDPsClinical keys DIC ↑ ↑ ↓ ↓ ↑ Shock Liver failure ↑ ↑ ↓ N/↓ N Jaundice Vitamin K def ↑ ↑ N N N Malabsorpt ion Sepsis without shock ↑ ↑ N N ↑/N Fever DD’s for DIC
  • 20.
  • 21.
    Causes a) Infectious-CMV,HCV,HIV b) Vaccination c)SLE d) APLA synd e) Drugs-quinidine,sulfonamide,heparin f) CVID g) Lymphoproliferative disorder
  • 22.
     Newly diagnosedITP-diagnosis to 3months  Persistent ITP-3to 12months of diagnosis  Chronic ITP->12months  Corticosteroid dependence ITP-need for ongoing or repeated administration of steroid to maintain platelet count in excess of 30x109 or avoid bleeding.  Severe ITP-bleeding at presentation of sufficient magnitude requiring treatment or occurrence of new bleeding symptoms requiring intervention  RefractoryITP-presence of severe ITP occuring after splenectomy
  • 23.
    MANAGEMENT GRADE 1 MINORBLEEDING FEW PETECHIAE(<100) <5 SMALL MUCOSAL BLEED Observation GRADE 2 MILD BLEEDING >100 PETECHIAE >5LARGE BRUISE(>3cm) NO MUCOSAL BLEEDS Observation Treatment in selected childred GRADE 3 MODERATE BLEEDING OVERT MUCOSAL BLEED MENORRHAGIA Intervention to reach grade1 or gr2 in selected children GRADE 4 MUCOSAL BLEEDS OR SUSPECTED INTERNAL BLEEDS INTERVENTION
  • 24.
    DRUGS APPROX RESPONSE RESPONSE TIME TOXICITY Iv antiD 50-75mcg/kg 50-77% Within 24hrs Headache,fever IVIG 0.8-1g/kg SD >80% 1-2days Fever Prednisolone 1-2mg/kg-14d or 4mg/kg 4days ¾ patient respond depending on dose 2-7days Gastritis Wait & watch 2/3 pt improve spontaneously within 6months Days to month -
  • 25.
    a)Children and adolscents PLTs <50,000/dl and bleeding  PLTs<50,000/dl and invasive procedure  PLTs <20,000/dl and marrow failure and hemorragic factors  PLTs any counts,but with PLTs dysfunction plus bleeding or on an invasive procedure
  • 26.
    Infants within thefirst 4 months of life:  PLTs <1lakh/dl and bleeding  PLTs<50,000/and an invasive procedure  PLTs<1lakh/dl and clinically unstable  PLTs any counts,but with PLTs dysfunction plus bleeding or on an invasive procedure
  • 27.
    Caused by anabsence or decreased amount of a procoagulant – 1. VIII -Hemophilia A affects ~ 1:5000 males 2. IX -Hemophilia B affects ~ 1:30000 males 3. XI –Hemophilia C – Rare /Ethnicity
  • 28.
    • Hallmark –HEMARTHROSIS •Easy bruising ,intramuscular hematomas,heamarthrosis • Life threatening condition like bleed into iliopsoas muscle,GI bleed
  • 29.
     Prolonged PTT(reducedlevel of 8 & 9)  Other screening test of hemostatic mechanism(platelet,bleeding time,PT)are normal.  Specific assay for factors VIII & IX confirms the diagnosis
  • 30.
     Dose calculationof r factor 8 and factor 9  Dose of factor 8 =%desired(rise in f8)xbody weight(kg)x0.5  Dose of factor 9 =%desired(rise in f9)xbody weight(kg)x1.4
  • 31.
     Factor 8concentrates- A)Plasma -derived factor VIII as well as factor VIII/von Willebrand factor (vWf) concentrates are prepared from cyroprecipitates and contain vWf and moderately enriched factorVIII. B) Recombinant factor -concentrates are prepared in animal cell cultures using biotechnological procedures Package Sizes-250/500/1000/1,500/2,000 U/package. storage temperature for concentrates is 2–8 °C
  • 32.
  • 33.
  • 34.
     VWF tethersplatelet to injured endothelium  VWF serves as carrier protein for factor 8  Stored in WEIBEL PALADE BODIES in endothelial cells  Facilitates its ability to bind to platelets by GP1b receptor.
  • 35.
     Easy bruisability Epistaxis or gingival bleeding  Menorrhagia  Post-surgical bleeding  Bleeding post-dental extraction
  • 36.
    Sub types ofVW Type 1 Partial quantitative deficiency of vWF 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 N - Decreased affinity for factor VIII Type 3 Complete deficiencey of vWF & Behave as Severe Hemophilia A
  • 37.
     VWf concentrates- Desmopressin sprays  antifibrinolytics
  • 38.
  • 39.
    factor XIII isresponsible for the crosslinking of fibrin to stabilize the fibrin clot, symptoms -delayed hemorrhage Typically, patients have trauma 1 day and then have a bruise or hematoma the next day. C/f  mild bruising, delayed separation of the umbilical stump beyond 4 wk in neonates,  poor wound healing,
  • 40.
    screening tests –forhemostasis are normal  The normal clot remains insoluble in the presence of 5M urea, whereas in a patient with XIII deficiency, the clot dissolves.  The half-life of factor XIII is 5-7 days  There is a heat-treated, lyophilized concentrate of coagulation factor XIII available to treat bleeding episodes or for prophylaxis.
  • 42.
  • 45.
    • Indication 1)to increaseoxygen delivery in an anemic patient 2)acute blood loss greater than 25-40% in blood volume. Dose-10ml/kg of RBC will increase Hb by 2.5-3g/dl Recommneded temp-33-35 C
  • 46.
     Can besingle or pooled whole blood  1U/10 kg=increase upto 50,000  Stored in mechanical agitator at 22 C upto 5days
  • 48.
     Freezing plasmaat -18 C within 8hrs of separation from whole blood  Contains all coagulation factors  Stored at -18C for 1yr  Prior to administration—thawed at 36-38C  10-15ml/kg over 30-120mins
  • 50.
     Contains fibrinogen(150mg/bag), factor8(80u/bag),VWf,factor 13  Highly concentrated form of plasma protein that settle down at bottom as ppte when frozen plasma is slowly thawed at 1-6 C  Dose-10-20 ml/kg
  • 51.