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Hematology

Hematology

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Published by: sarguss14 on Feb 20, 2009
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PEDIATRICS
Hematology Part 2
DR. ALABASTRO

3rd Shifting/2/2/09
\u201cSMV\u201d
BLEEDING
COMPONENTS OF HEMOSTASIS

1. Vessel wall
2. Platelets
3. Coagulation protein
4. Anticoagulant protein
5. Fibrinolytic system

HOMEOSTASIS
Blood vessel injury
\u2192
VASOCONSTRICTION
\u2192 1.
VASCULAR PHASE
(1st stimulus)
Platelet
platelet
\u2192 Platelet plug \u21922.
PLATELET PHASE
adhesion
aggregation
Prothrombin
Tissue
\u2192
\u2190 Plasma Factors
Thromboplastin
(INTRINSIC)
(EXTRINSIC)Fibrinogen
Fibrin

Plasma thromboplastin time: intrinsic pathway, common pathway
Coagulation time: intrinsic pathway, common pathway
Prothrombin tim: extrinsic pathway, common pathway

Thrombin time: common pathway
ANTICOAGULANTS
1. Tissue factor pathway inhibitor (TFPI)
1. Regulates tissue factor (TF) and Factor VIIa
2. Protein C and Protein S (P-C/S)
2. Regulate Factors VIII and V
3. Antithrombin III (AT-III)
3. Regulates Factor Xa and thrombin (Factor IIa)
MECHANISMS OF HOMEOSTASIS
Endothelial Cells \u2013 secretes substances that:

1.Repel platelets: PGI2, ADP, NO
2.Initiate coagulation: fibrinectin, vWF
3.Initiate fibrin dissolution: tissue plasminogen

activator
4.Catalyze inhibition of thrombin:heparin,
thrombomodulin
5.Inhibit initiation of fibrin dissolution:tissue
plasminogen activator inhibitor
Platelets:
1.Release: ADP, serotonin, calcium, fibrinogen,
vWF, Factor V, platelet-derived growth factor
2.Aggregate and increase mass of hemostatic
plug
3.Mediate blood vessel constriction (serotonin)
4.Neutralizehep arin
Plasma Coagulation and Fibrinolysis Factorsa re
initiated by substances secreted and released by
endothelial cells.
Vitamin K dependent factors are activated in platelet
phospholipid surfaces.
Thrombin promotes platelet aggregation and release

reactions; generates a biofeedback to form more thrombin at a faster rate; activates factor XIII and insolubilizes the fibrin network.

EVALUATION OF A HEMOSTATIC DEFECT
1. Detailed History
-
Hemorrhage: site, severity, duration, onset
(spontaneous, after trauma)
-
Thrombosis/emboli: family history
2. Complete Physical Examination
-
Muscles, mucocutaneous, joints
3. Complete Blood Count
-
Platelet count
4. Coagulation Tests

MARY YVETTE ALLAIN TINA RALPH SHERYL BART HONEY PIPOY TLE JAM CECILLE DENESSE VINCE HOOPS CES XTIAN LAINEY RIZ KIX EZRA GOLDIE BUFF MONA AM MAAN ADI KC PENG
KARLA ALPHE AARON KYTH ANNE EISA KRING CANDY ISAY MARCO JOSHUA FARS RAIN JASSIE MIKA SHAR ERIKA MAQUI VIKI JOAN PREI KATE BAM AMS HANNAH MEMAY PAU RACHE
ESTHER JOEL GLENN TONI

ADP
TXA
3.P LASMA
PHASE
Shar, Maqui & Viki
Hematolgy Part 2
-
BT, PTT, PT, thrombin time
5. Peripheral Smear
6. Bone Marrow
-
Status of megakaryocytes
Study
Purpose
Clinical
Significance
Bleeding time

Measures
platelets and
vascular
function

Prolonged in:

ITP,
thrombocytopat
hy,

von
Willebrand\u2019s,
aspirin
ingestion,
anticoagulant,
uremia

Platelet count

Assesses
platelet
concentration

Decrease
in:
ITP,
bone
marrow
malignancies,
drugs
Elevated
in:

early
myeloproliferativ
e disorders

Clot retraction

Assesses
platelet
adequacy to
form fibrin clot

Poor
in:
thrombocytopeni
a
and
polycythemia;
lysis of clot in
fibrinolysis

Lee-White
clotting time
(coagulation)

Assesses
coagulation
mechanism

Prolonged in:
severe
deficiencies
of
coagulation
factors
Prothrombin
time (PT)

Measures
extrinsic and
common
coagulation
pathways

Prolonged in:
deficiencies

of Factors VII and X and fibrinogen, excess

dicumarol,
severe

liver disease and vit.K deficiency

Activated partial
thromboplastin
time (aPTT)

Measures
intrinsic and
common
coagulation
pathways

Prolonged in:
deficiencies

of Factors VIII to XII and fibrinogen, anticoagulant

therapy,
liver
disease,
DIC,
vit.K deficiency
Thrombin time
(TT)

Measures
fibrinogen to
fibrin formation

Prolonged in:
low
fibrinogen
levels, DIC, liver

disease,
anticoagulant
therapy,
dysproteinemias

Thromboplastin
generation time
Measures ability
to form
Prolonged in:
thrombocytopeni
(TGT)
thromboplastin
a
with
deficiencies

of Factors VIII to XII,

anticoagulants
BLEEDING DISORDERS
Vascular Factors
1. Trauma or other physical cause
2. Infectious diseases
3. Drugs, chemicals, toxins
4. Scurvy
5. Allergic or anaphylactoid purpura
6. Irradiation
7. Metabolic: Cushing syndrome
8. Hereditary telangiectasia
9. Ehlers-Danlos syndrome
Intravascular Factors
1. Thrombocytopenia
-
ITP, secondary (infections, drugs, toxins,
irradiation, neoplasm, etc.)
2. Qualitative Platelet Disorder
-

Characterized by easy bruising, epistaxis, oozing after dental extraction, spontaneous petechiae, Glanzmann thrombasthenia

3. Inherited Deficiencies of Coagulation
-
Hemophilias, deficiency of other factors
4. Acquired Clotting Factor Deficiencies
-
DIC, Vitamin K deficiency, liver disease,
uremia, congenital heart disease
IDIOPATHIC THROMBOCYTOPENIC PURPURA
-
Due to autoantibodies, drug-dependent antibodies
or alloantibodies
-

Characterized by: thrombocytopenia, shortened platelet survival, presence of antiplatelet antibody in the plasma, increased megakaryocytes in the bone marrow

-May beacute (returns to normal within 6 months),
chronic (beyond 6 months), recurrent
-
Incidence: between 1 and 8 years, both sexes
-
Predisposing factors: infection within preceding 1-4
weeks
-
Signs and symptoms:
o
Ecchymoses or purpura: anterior surface of
lower extremities and bony prominences
o
Petechiae: subconjunctivae, buccal mucosa,
soft palate, skin
o
Bleeding: nose, gums, mucus membranes,
GIT or kidneys
o
PE not significant: pallor, splenomegaly,
cervical lymphadenopathy rare
-
Laboratory:
o
Platelet count:<100,000/mm3
o
Blood smear: normal, mild eosinophilia,
anemia
o
Bone marrow: \u2191 megakaryocytes, \u2191
eosinophils
Shar, Maqui & Viki
Hematolgy Part 2
o
Coagulation profile: abnormal bleeding
time, poor to absent clot retraction, normal PT,
PTT
and
fibrinogen
level,
defective
prothrombin consumption test
-
Diagnostic Criteria:
o
Clinical exam: purpura with essentially
normal PE
o
Platelet
and
blood
smears:
thrombocytopenia only
o
Bone marrow: normal to \u2191megakaryocytes
o
Exclusion
of
secondary
causes
of
thrombocytopenia:

hypersplenism, microangiopathic hemolytic anemia, DIC, drug- induced, SLE, viral infections

-Treatment:
o
No treatment when platelet count is
>35,000/mm3 and asymptomatic
o
Contact sports avoided
o
Steroids: prednisone (60 mg/day); reduced by
10 mg at 5-7 day intervals for 2-3 weeks; stop
at end of course
o
Repeat 4-week course after a month if no
response
o
High dose IV gamma globulin: fewer days
of bleeding and faster response
o
Combination of steroid and IVIG
o
Anti-Rh D therapy
o
Platelet transfusion
o
Splenectomy for chronic and life-threatening
immunosuppressants
HEMOPHILIA
-
Both A and B are x-linked recessive

-A: procoagulant factor VIII
-B: procoagulant factor IX
-Indistinguishable clinical manifestations:

Bleeding occurs in a close space; hallmark is
HEMARTHROSIS
-Detection of carriers: DNA analysis; FVIII/FIX
activity; direct gene mutation analysis
-
Symptoms:
o
Severe:
spontaneous
hemarthrosis;
deep tissue
o
Moderate: gross bleeding following mild
to moderate trauma; some hemarthoses;
seldom spontaneous
o
Mild: severe hemorrhage only if
moderate to severe trauma or surgery
o
High risk carrier females:gyne cologic
and obstetric hemorrhages
-Laboratory:
o
APTT: 2-3x upper normal
o
Platelet count, bleeding time,
prothrombin time and thrombin
time:norm al
o
Specific factor assay
o
Quantitative Bethesda assay for
inhibitors
-
Treatment
o
Replacement:FVIII/FIX
o
Release of FVIII:D DAV P
(desmopressin) intranasal \u2013 for mild
hemophilia
o
DDAVP ineffective for hemophilia B and
moderate hemophilia
o
Supportive: avoid trauma and NSAIDS;
give hep B vaccine
-
Complications:
o
Chronic joint destruction:
arthropathy; high plasma levels of
clotting factors; gene therapy
o
Transfusion-transmitted infectious
diseases: HIV, Hepatitis; use of
recombinant products
o
Development of inhibitor to FVIII or
FIX: continued regular transfusions,
desensitization; activated prothrombin
complex concentration or FVIIa
VITAMIN K DEFICIENCY
-Full term: low levels of FII, VII, IX, X; low intake
(lower in breast milk)
-Prematures: low stores and factors; response
to vitamin K slow (immature liver)
-Between 2nd and 4th day of life
-Older infants and children:malabsorption;
antibiotics; hepatocellular; drugs
DIC
-Treatment:
o
Control or reversal of trigger:
hypoxia, acidosis, tissue necrosis, shock,
endothelial damage
o
Blood components:platel ets,
cryoprecipitate, fresh frozen plasma
o
Heparin + clotting factor and platelet
replacement
DIC
Trigge
r

Hypoxia,
acidosis, tissue
necrosis, shock,
endothelial
damage

Thrombin
generation
Fibrinemi
a

Consumption of
platelets &
coagulation
factors (V, VIII,
fibrinogen,
prothrombin)

Microvascular
thrombosis

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