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PEDIATRICS

Hematology Part 2
DR. ALABASTRO
3rd Shifting/2/2/09
“SMV ”

BLEEDING Thrombin time: common pathway


COMPONENTS OF HEMOSTASIS
ANTICOAGULANTS
1. Vessel wall
2. Platelets 1. Tissue factor pathway inhibitor (TFPI)
3. Coagulation protein 1. Regulates tissue factor (TF) and Factor VIIa
4. Anticoagulant protein 2. Protein C and Protein S (P-C/S)
5. Fibrinolytic system 2. Regulate Factors VIII and V
3. Antithrombin III (AT-III)
HOMEOSTASIS 3. Regulates Factor Xa and thrombin (Factor IIa)

Blood vessel injury → VASOCONSTRICTION → 1. MECHANISMS OF HOMEOSTASIS


VASCULAR PHASE
(1st stimulus) Endothelial Cells – secretes substances that:
1. Repel platelets: PGI2, ADP, NO
ADP
Platelet TXA platelet → Platelet plug → 2. 2. Initiate coagulation: fibrinectin, vWF
PLATELET PHASE 3. Initiate fibrin dissolution: tissue plasminogen
adhesion aggregation
activator
4. Catalyze inhibition of thrombin: heparin,
thrombomodulin
Prothrombin 5. Inhibit initiation of fibrin dissolution: tissue
Tissue → ← Plasma Factors
plasminogen activator inhibitor
Thromboplastin (INTRINSIC)
3. PLASMA
(EXTRINSIC) PHASE Platelets:
Fibrinogen Fibrin 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. Neutralize heparin
Plasma Coagulation and Fibrinolysis Factors are
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
Plasma thromboplastin time: intrinsic pathway, common pathway - Muscles, mucocutaneous, joints
Coagulation time: intrinsic pathway, common pathway 3. Complete Blood Count
Prothrombin tim: extrinsic pathway, common pathway - 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
Shar, Maqui & Viki
Hematolgy Part 2

- BT, PTT, PT, thrombin time a with


5. Peripheral Smear deficiencies of
6. Bone Marrow (TGT) thromboplastin Factors VIII to
- Status of megakaryocytes XII,
anticoagulants

BLEEDING DISORDERS
Clinical
Study Purpose
Significance Vascular Factors
Prolonged in: 1. Trauma or other physical cause
ITP, 2. Infectious diseases
thrombocytopat 3. Drugs, chemicals, toxins
Measures
hy, von 4. Scurvy
platelets and
Bleeding time Willebrand’s, 5. Allergic or anaphylactoid purpura
vascular
aspirin 6. Irradiation
function
ingestion, 7. Metabolic: Cushing syndrome
anticoagulant, 8. Hereditary telangiectasia
uremia 9. Ehlers-Danlos syndrome
Decrease in:
ITP, bone Intravascular Factors
marrow 1. Thrombocytopenia
Assesses malignancies, - ITP, secondary (infections, drugs, toxins,
Platelet count platelet drugs irradiation, neoplasm, etc.)
concentration Elevated in: 2. Qualitative Platelet Disorder
early - Characterized by easy bruising, epistaxis,
myeloproliferativ oozing after dental extraction, spontaneous
e disorders petechiae, Glanzmann thrombasthenia
Poor in: 3. Inherited Deficiencies of Coagulation
Assesses thrombocytopeni - Hemophilias, deficiency of other factors
platelet a and 4. Acquired Clotting Factor Deficiencies
Clot retraction - DIC, Vitamin K deficiency, liver disease,
adequacy to polycythemia;
form fibrin clot lysis of clot in uremia, congenital heart disease
fibrinolysis
Prolonged in: IDIOPATHIC THROMBOCYTOPENIC PURPURA
Lee-White Assesses severe
clotting time coagulation deficiencies of - Due to autoantibodies, drug-dependent antibodies
(coagulation) mechanism coagulation or alloantibodies
factors - Characterized by: thrombocytopenia, shortened
Prolonged in: platelet survival, presence of antiplatelet antibody
deficiencies of in the plasma, increased megakaryocytes in the
Measures Factors VII and X bone marrow
Prothrombin
extrinsic and and fibrinogen, - May be acute (returns to normal within 6 months),
common excess chronic (beyond 6 months), recurrent
time (PT)
coagulation dicumarol, - Incidence: between 1 and 8 years, both sexes
pathways severe liver - Predisposing factors: infection within preceding 1-4
disease and vit.K weeks
deficiency - Signs and symptoms:
Prolonged in: o Ecchymoses or purpura: anterior surface of
deficiencies of lower extremities and bony prominences
Measures
Factors VIII to XII
Activated partial intrinsic and
and fibrinogen,
o Petechiae: subconjunctivae, buccal mucosa,
thromboplastin common soft palate, skin
anticoagulant
time (aPTT) coagulation o Bleeding: nose, gums, mucus membranes,
therapy, liver
pathways
disease, DIC, GIT or kidneys
vit.K deficiency o PE not significant: pallor, splenomegaly,
Prolonged in: cervical lymphadenopathy rare
low fibrinogen - Laboratory:
Measures levels, DIC, liver o Platelet count: <100,000/mm3
Thrombin time
fibrinogen to disease,
(TT) o Blood smear: normal, mild eosinophilia,
fibrin formation anticoagulant
therapy, anemia
dysproteinemias o Bone marrow: ↑ megakaryocytes, ↑
Thromboplastin Measures ability Prolonged in: eosinophils
generation time to form thrombocytopeni
Shar, Maqui & Viki
Hematolgy Part 2

o Coagulation profile: abnormal bleeding o Quantitative Bethesda assay for


time, poor to absent clot retraction, normal PT, inhibitors
PTT and fibrinogen level, defective - Treatment
prothrombin consumption test o Replacement: FVIII/FIX
- Diagnostic Criteria: o Release of FVIII: DDAVP
o Clinical exam: purpura with essentially (desmopressin) intranasal – for mild
normal PE hemophilia
o Platelet and blood smears: o DDAVP ineffective for hemophilia B and
thrombocytopenia only moderate hemophilia
o Bone marrow: normal to ↑megakaryocytes o Supportive: avoid trauma and NSAIDS;
o Exclusion of secondary causes of give hep B vaccine
- Complications:
thrombocytopenia: hypersplenism,
microangiopathic hemolytic anemia, DIC, drug- o Chronic joint destruction:
induced, SLE, viral infections arthropathy; high plasma levels of
clotting factors; gene therapy
- Treatment:
o No treatment when platelet count is o Transfusion-transmitted infectious
diseases: HIV, Hepatitis; use of
>35,000/mm3 and asymptomatic
recombinant products
o Contact sports avoided
o Development of inhibitor to FVIII or
o Steroids: prednisone (60 mg/day); reduced by FIX: continued regular transfusions,
10 mg at 5-7 day intervals for 2-3 weeks; stop desensitization; activated prothrombin
at end of course complex concentration or FVIIa
o Repeat 4-week course after a month if no
response VITAMIN K DEFICIENCY
o High dose IV gamma globulin: fewer days
of bleeding and faster response - Full term: low levels of FII, VII, IX, X; low intake
o Combination of steroid and IVIG (lower in breast milk)
o Anti-Rh D therapy
o Platelet transfusion - Prematures: low stores and factors; response
o Splenectomy for chronic and life-threatening to vitamin K slow (immature liver)
immunosuppressants - Between 2nd and 4th day of life
- Older infants and children: malabsorption;
antibiotics; hepatocellular; drugs
HEMOPHILIA
DIC
- Both A and B are x-linked recessive
- A: procoagulant factor VIII - Treatment:
- B: procoagulant factor IX o Control or reversal of trigger:
- Indistinguishable clinical manifestations: hypoxia, acidosis, tissue necrosis, shock,
Bleeding occurs in a close space; hallmark is endothelial damage
HEMARTHROSIS o Blood components: platelets,
- Detection of carriers: DNA analysis; FVIII/FIX cryoprecipitate, fresh frozen plasma
activity; direct gene mutation analysis o Heparin + clotting factor and platelet
- Symptoms: replacement
o Severe: spontaneous hemarthrosis;
deep tissue
o Moderate: gross bleeding following mild DIC Hypoxia,
to moderate trauma; some hemarthoses; acidosis, tissue
seldom spontaneous necrosis, shock,
endothelial
o Mild: severe hemorrhage only if Trigge
damage
moderate to severe trauma or surgery r
o High risk carrier females: gynecologic
and obstetric hemorrhages Thrombin
- Laboratory: generation
o APTT: 2-3x upper normal Consumption of
Fibrinemi platelets &
o Platelet count, bleeding time, a coagulation
prothrombin time and thrombin factors (V, VIII,
time: normal fibrinogen,
o Specific factor assay Microvascular
prothrombin)
thrombosis
Shar, Maqui & Viki
Hematolgy Part 2

Inactive Active

Preservation of Proteolysis of
fibrin fibrin ONCOLOGY

LEUKEMIA
Organ Hemorrhagic
damage diathesis
• ETIOLOGY: Radiation, chemicals, drugs, genetic
Component Vitamin K Liver DIC
disease
• SYMPTOMS: fever, bleeding, bone pain,
RBC normal target Fr, Burr, lymphadenopathy, splenomegaly,
schista hepatosplenomegaly
PTT prolong prolong prolong • SIGNS: anemia, neutopenia, thrombocytopenia
PT prolong prolong prolong • DIAGNOSIS:
Fib. Spl Normal Normal or Marked inc 1. CBC: Hgb ( mod-marked ↓); WBC (low, normal
inc or ↑); ↓platelets
Platelets Normal Normal Decrease 2. BLOOD SMEAR: normocytic, normochromic,
Decrease II, VII, IX, X I, II, V, VII, I, II, V, VII, anemia; blasts; occ. Eosionophilia
factors IX, X VIII 3. BONE MARROW: blasts; absent megakaryocyte
4. CXR : mediastinal mass
JAUNDICE 5. COAGULATION PROFILE: ↓factors in AML
6. CSF: CNS leukemia
- Unconjugated: Hemolytic • DIFFERENTIAL DIAGNOSIS:
o Hemolytic disease of the newborn 1. Idiopathic thrombocytopenic purpura
o Erythrocyte membrane abnormality 2. Aplastic anemia
o Erythrocyte enzyme deficiency 3. Mononucleosis
o Alpha thalassemia 4. Juvenile rheumatoid arthritis
o Sickle cell anemia 5. Leukemoid reaction
o Vitamin K overdose 6. Malignant diseases that invade bone marrow
o Oxytocin induction • TREATMENT
- Unconjugated: Polycythemia 1. INDUCTION: (4-6wks)
o Placental hypertransfusion: twin-  vincristine (IV/wk)
twin; maternal-fetal; delayed cord  asparaginase (IM, biweekly)
clamping  prednisone (PO/day)
o Placental insufficiency: SGA; 2. INTRATHECAL: (wkly x 6; q 8 wks x 2 years)
postmaturity; toxemias; previa  MTX
o Endocrine: CAH; thyrotoxicosis;  Hydrocortisone
maternal diabetes  cytarabine
o Miscellaneous: Down syndrome; 3. CONTINUATION: (2-3yrs)
hyperplastic visceromegaly  6-MP (PO/day)
- Unconjugated: Other causes  MTX (PO, IV, IM/wk)
 Prednisone/ vincristine
o Hematoma: cephalhematoma, etc.
o Conjugation defects: Crigler-Najjar; NEUROBLASTOMA
Gilbert’s disease
o Metabolic: hypothyroidism; DM; • Most common solid tumor outside CNS
galactosemia • Diagnosed before age 5
o Gut obstruction: pyloric stenosis; • Any site of SNS: abdomen (adrenal,
duodenal atresia retroperitoneal)
• SX: hard, fixed, abdominal mass, nerve root
compression, bone pain, “dancing eye, dancing
feet” syndrome (ataxia & opsomyoclonus)
Hemoglob
in • DIAGNOSIS:
1. XRAY, CT, MRI : mass with calcification/
hemorrhage
High Normal Low 2. HVA & VMA : elevated
3. BIOPSY
4. BONE MARROW: neuroblast
Polycythem Conjugation Examine infant
ia defects
Metabolic
defects
Gut obstruction
Shar, Maqui & Viki
Hematolgy Part 2

5. BONE SCAN: metastasis


6. CYTOGENETIC & MOLECULAR
MARKERS:prognosis (MYCN, 1p deletion)
• TREATMENT
1. SURGERY: determined by imaging studies
& relationship of tumor to other organs
2. RADIATION: stage 3 tumors
3. CHEMOTHERAPY: cisplastin, doxorubicin,
vincristine, cyclophosphamide
4. SUPPORTIVE: blood, infections,
complications

WILMS TUMOR

• Associated with genitourinary anomalies


• Solitary growth in any part of kidney
• Median age on diagnosis: 3 years
• SYMPTOMS: asx abdominal (mass smooth, firm,
rarely crosses midline) hypertension, hematuria
• DIAGNOSIS: UTZ, CT scan
• TREATMENT
1. SURGERY: evaluate metastasis
2. CHEMOTHERAPY: vincristine, actinomycin.
doxorubicin
3. RADIATION: dose modified to dec incidence
of scoliosis
4. PULMONARY IRRADIATION: +3-drugs (stage
4)
5. PREOPERATIVE THERAPY: for bilateral
tumors

RETINOBLASTOMA

• Familial (multifocal & bilateral)


• Nonfamilial (unifocal & unilateral)
• Outgrows blood supply of retina: necrosis &
calcification
• SYMPTOMS: Leukocoria( white papillary reflex)
Strabismus, orbital inflammation,
pupil
irregular and pain
• DIAGNOSIS: opthalmoscopy; UTZ,CT, MRI
• TREATMENT:
1. Enucleation:
 unilateral ( no potential for
vision loss)
 bilateral (more affected eye)
2. Laser Photocoagulation:cryotherapy
3. Chemotherapy: carboplastin, vincristine,
etoposide
4. Brachytherapy: if nonresponsive

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