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    By: Dr. Sem Ravy Medical Doctor at ICU, Jayavarman VII Hospital

    Uploaded by

    Ravy Sem

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    © All Rights Reserved
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    By: Dr. Sem Ravy Medical Doctor at ICU, Jayavarman VII Hospital

    Uploaded by

    Ravy Sem
    3 views34 pages

    Original Title

    ITP

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    Copyright:

    © All Rights Reserved
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    3 views34 pages

    By: Dr. Sem Ravy Medical Doctor at ICU, Jayavarman VII Hospital

    Uploaded by

    Ravy Sem

    Copyright:

    © All Rights Reserved
    Download as PDF, TXT or read online from Scribd
    Download as pdf or txt
    of 34

    By: Dr.

    Sem Ravy
    Medical Doctor at ICU, Jayavarman VII Hospital.
    2

    I. Objective
    II. Introduction
    III. Pathogenesis
    IV. Epidemiology
    V. Etiology
    VI. Clinical Features and Diagnosis
    VII.Management
    VIII.Case Study
    3

    ´ Managemet ITP of children in ICU:


    Ø Counseling/advice
    Ø Corticotherapy +++

    Ø IVIG
    Ø Anti-D therapy
    Ø Splenectomy
    Ø Thrombopoietin receptor agonists
    4

    ´ ITP : disorder caused by antiplatelet antibodies, leading to


    accelarated destruction, and inhibition of the production of platelets.
    Ø Idiopathic thrombocytopenic purpura
    Ø Immune thrombocytopenic purpura
    Ø Auto-immune thrombocytopenic purpura

    ´ Life time of Platelet : 7-10 days


    ´ Platelet : 150.10⁹/L - 400.10⁹/L
    ´ Trombocytopenia < 150.10⁹/L
    5

    ´ Acute ITP: < 3 months from the initial diagnosis.


    ´ Persistent ITP: 3-12 months from the initial diagnosis.
    ´ Chronic ITP: > 12 months from the intial diagnosis.

    ´ Trombocytopenia resolved -/+ treatment < 6 months => 80%


    ´ Trombocytopenia unresolved -/+ treatment > 12 months => 20%
    6

    ´ Autoantibody directed against the platelet surface develops =>


    thrombocytopenia.

    ´ After binding of the antibody to the platelet surface => circulating


    antibody-coated platelets, then recognized by the Fc receptor on
    splenic macrophages => ingested, and destroyed.
    7
    8

    ´ The most common cause of acute illness of thrombocytopenia in


    an otherwise well child.
    ´ Approximately: 7.2 - 9.5/100 000 childrent between 1-14y of age.
    ´ 1-4 weeks after exposure to a common viral infection (50-65% of
    cases of childhood ITP).
    ´ Peak age: 2-5 years old..
    ´ Sex ratio: male:female = 1:1
    ´ Seasonal variation: more often in late winter and early spring, after
    the peak season of viral respiratory illness.
    9

    ´ Primary ITP: Idiopathic: thrombocytopenia + absence of other


    causes or disorders associated (80%). +++
    ´ Secondary ITP: thrombocytopenia + other causes or disorders
    associated (20%):
    Ø Post infection: HIV, HCV, CMV, EBV, varicella, rubella, mumps, measles, H.
    Pylori…
    Ø Drugs induced: chemotherapy, ionizing radiation, Chloramphenicol, Estrogen,
    sulfonamide, quinidine, quinine, Cabamazepine, Valproic acid, digoxin…
    Ø Post transfusion
    Ø Lymphoproliferative disorders
    Ø Immune dificiency disorder
    Ø SLE
    Ø Anti phospholipid syndrome
    10

    ´Severity of bleeding in ITP is on the basis of symptoms and


    signs, but not platelet count:
    Ø No symptoms
    Ø Mild symptoms: bruising, purpura, petechiae, minor epistaxis =>
    very little interference with daily living.
    Ø Moderate: more severe skin and mucosal lesions, some epistaxis,
    menorrhagia, gum bleeding => more trouble with daily living.
    Ø Severe: bleeding episodes (<3%): => hospitalization in ICU:
    § Menorrhagia, epistaxis, melena => anemia => requiring blood transfusion,
    symptoms interfering seriously with the quality of life.
    § Neurological signs => ICH (0.1-0.8% of ITP): is the most serious.
    11
    12

    ´3 keys diagnostic criteria:


    ØIsolated thrombocytopenia + normal CBC and blood
    smear (particularly RBC and WBC morphology).
    ØAbsence of hepatosplenomegaly, lymphadenopathy,
    bone or joint pain and other congenital anomalies.
    ØPlatelet response to ITP therapy (Corticosteroids, IVIG,
    Anti-D).
    13

    ´Laboratory Findings:
    Ø CBC: +++
    ´Platelet count < 100.10⁹/L
    ´WBC, RBC and other differential count: normal
    ´Hb and Ht: maybe low if profuse bleeding <= severe thrombocytopenia
    < 20.10⁹/L.

    Ø Bone marrow aspiration/biopsy: BMA? Malignancy?...


    Ø Coombs test: Evans Sd?, HUS?...
    Ø PT, PTT, Fibrinogen, D-Dimer: Coagulopathies? …
    Ø BC, CRP: if persistent fever: sepsis?
    14

    ´Radiography finding:
    Ø Brain CT-Scan or MRI:
    ´Neurological signs with or without severe
    thrombocytopenia => ICH?
    15

    ´Goals:
    ØTo increase platelet count => prevent severe bleeding.
    ØTo alleviate fatigue or difficulty with activities of daily
    living.
    16

    ´When to treat ITP?

    Ø If platelet count > 30.10⁹/L + No sign of bleeding => no medical


    treatment, counseling of parents and careful observation
    within 6 to 12 months.
    Ø If platelet count > 30.10⁹/L + Bleeding, or platelet count <
    30.10⁹/L => Medical treatment needed.
    17

    ´ Counseling/advice:
    ØAvoid contact sport
    ØAvoid IM injection
    ØAvoid NSAID
    ØAbout 60%-80% of patients recoverd within 6-12 months,
    without any treatment.
    Ø If the child has bleeding signs => Come to see the doctor.
    18

    ´ Corticotherapy: +++
    Ø Action of corticosteroids:
    ´ Inhibit phagocytosis of antibody coated platelet.
    ´ Supress activation of T-cells driving the auto immune respone.
    ´ Inhibit antibody produced by B-lymphocytes.
    ´ Improve platelet production.
    ´ Improve capillary resistance => reduce bleeding.
    19

    ´ Corticotherapy: +++
    Ø Prednisone:
    ´Dose: 1-2mg/kg/d (max: 60mg/d), orally for 2-4 weeks, then
    tapering every week after platelet response occur.
    ´The initial response rate is 50%-90%.
    ´Increasing platelet: after 2-3 weeks.
    Ø Methyl prednisolone:
    ´Dose: 30mg/kg/d (max: 1000mg/kg/d) (IV) for 3 days, then
    20mg/kg/d for 3 days, and then tapering every week.
    20

    ´ Corticotherapy: +++
    Ø Side effects:
    ´ Infection
    ´ Weight gain
    ´ Gastritis/ulcers
    ´ Moon facies
    ´ Fluid retention
    ´ Hypertension
    ´ Glucose intolerance => diabetes
    ´ Osteoporosis/growth failure …
    21

    ´ IVIG:
    ØAction: Downregulation of Fc-mediated phagocytosis of
    antibody coated platelet.
    ØDose: 0.8-1g/kg/d, 1 dose/day, for 1-3 days
    ØEffective: 90%-95%
    ØIncreasing platelet: after 24h-48h.
    ØSide effects: headache, nausea/vomiting, fever,
    anaphylaxis, aseptic meningitis (rare) …
    22

    ´ IV Anti-D: for Rhesus (+) patients


    ØAction: anti-D bind with RBC => RBC antibody complexe,
    bind to macrophage Fc receptor and interferes with
    dysfunction.
    ØDose: 50-75mcg/kg, single dose.
    ØEffective: 80%-90%
    ØIncreasing platelet: after 48h-72h.
    ØSide effects: fever/chill, headache, nausea/vomiting,
    intravascular haemolysis, anaphylaxis (rare) …
    23

    ´Other treatment:
    Ø Antibiotic
    Ø Tagamet/Nexium
    Ø Tranexamic acid
    Ø Analgesics (Perfalgan, Morphine…)
    Ø Blood transfusion
    24

    ´Emergency therapy:
    Ø Methyl prednisolone: 30mg/kg/d (IV), for 1-3 days,
    Ø IVIG 1g/kg/d for 2-3 days, with or without,
    Ø Anti-D 75mcg/kg, 1 dose,
    Ø Platelet transfusion.
    25

    ITP

    Mild/Moderate Severe
    Symptoms bleeding/ICH

    Prednisolone 1-2mg/kg/d, Methyl prednisolone


    orally for 2-4 weeks, then 30mg/kg/d (IV), for 3d,
    tapering every week after 20mg/kg/d for 3d, then
    platelet response tapering every week, and
    followed by Prednisolone
    orally
    26 Case study – 01
    ´ A 1y old, boy, was admitted on 06-12-2021, for FD2 + Anemia, to ICU3, then
    transferred to C4, and discharged home on 22-12-2021.
    Ø Physical examination: (06-12-2021)
    § Fever : 39C
    § Pallor
    § Ecchymosis
    § No hepatosplenomegaly
    § No melena
    Ø CBC:
    § WBC: 21,3 .10⁹/L
    § Ht: 6%
    § PLT: 46 .10⁹/L
    § CRP: 113.2, BC: (-)
    § PT: 17 Sec, PTT: 30 Sec
    27 Case study – 01

    ´ Management:
    Ø Solu-Medrol (start on 08-12-2021), then followed by Prednisolone orally
    Ø Ceftazidime
    Ø Nexium
    Ø Tranexamic acid
    Ø Konakion
    Ø Panadol
    Ø Blood transfusion
    28 Case study – 01

    ´ On 15-12-2021 (1 week after treatment )


    Ø Clinical sign: better
    Ø CBC:
    § WBC: 11,5 .10⁹/L
    § Ht: 27%
    § PLT: 50 .10⁹/L

    § On 20-01-2022 (6 weeks after treatment)


    Ø Clinical sign: ecchymosis, pallor
    Ø CBC:
    § WBC: 9,3 .10⁹/L
    § Ht: 26%
    § PLT: 94 .10⁹/L
    29 Case study – 02
    ´ A 12y old, girl, was admitted on 31-01-2022, for G. ecchymosis, to C4, then
    transferred to ICU3, because of severe anemia + severe headache.
    Ø Physical Examination: (on 31-01-2022)
    § Consciousness
    § Pallor
    § Severe headache + Vomiting
    § Ecchymosis, Gum bleeding
    § No hepatosplenomegaly
    § No melena
    Ø CBC:
    § WBC: 9,7 .10⁹/L
    § Ht: 17%
    § PLT: 70 .10⁹/L
    § CRP: 12,4 ; BC (-)
    § PT: 15 sec, PTT: 28 sec
    30 Case study – 02

    ´ Brain MRI: (on 01-02-2022)


    Ø ICH at left occipital lobe about 3 x 4.1 cm
    31 Case study – 02

    ´ Management :
    Ø Solu-Medrol
    Ø Mesporine
    Ø Tranexamic acid
    Ø Tagamet
    Ø Konakion
    Ø Panadol / Morphine
    Ø Blood transfusion
    32 Case study – 02

    ´ On 09-02-2022 (8 days after treatment)


    Ø Clinical signs: better Ø Brain MRI
    Ø CBC:
    § WBC: 13,2 .10⁹/L
    § Ht: 30%
    § PLT: 122 .10⁹/L
    33 Case study – 02

    ´ On 15-02-2022 (2 weeks after treatment):


    Ø CBC:
    § WBC: 15,2 .10⁹/L
    § Ht: 29%
    § PLT: 268 .10⁹/L

    ´ Discharged home: 21-02-2022


    ´ Last appointment: 18-03-2022
    Ø CBC:
    § WBC: 8,6 .10⁹/L
    § Ht: 37%
    § PLT: 374 .10⁹/L
    34

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