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  • Chief complaint : 2

    Case 2 . .

  • HistoryPresent illness :

    4 . vaccine DPT-HB

    2 . 4 . CBC Plt. 7,000 ..

  • General appearance : A Thai infant , active crying with tear vital signs : BT 36.5 C , PR 84 bpm , RR 22 bpm , BP 90/60 mmHg HEENT : AF 1x1 cm. , no bulging , not pale , no jaundice , no petechial in oral mucosa

    Abdomen : no distention ,normoactive BS, soft , not tender, no guarding , liver and spleen cant be palpable.

    Ext : Petechiae at both legs , hand , face , no ecchymosis , no edema all extremities.

    .

  • Lab ( ..)CBC :Hb 9.5 Hct 29% , WBC 9580 /mm3 ,platelet 7,000 /mm3 ,PMN 9 % , Lymphocyte 81 %, MCV 75.4 , MCH 24.9

  • Past History

    - - (

    )- -

  • HistoryFamily history :

    -

    Personal history :

    - C/S due to PIHApgar score 9,10 2,755

    - - 5.5 . , 65 .-

  • Physical examinationVital signs : BT 37.0 C, PR 156 bpm,full RR 44 /min BP 76/50 mmHg ,

    Body weight 5.5 kg,Height 65 cmGeneral appearance : A Thai infant, good consciousness, no

    drowsinessHEENT : AF 2x1 cm. ,not pale conjunctivae,anicteric sclerae, pharynx

    and tonsils were not injected, CLN & SCLN were impalpableLungs : equal breath sound, no retraction, no adventitious sound Heart : normal S1S2, no murmurAbdomen : soft, not tender, active bowel sound, liver and spleen

    were impalpable, kidneys were impalpableExtremities : Petechial both legs, hand, face, no ecchymosis, no edema,

    no deformitiesNS : Active , Pupil 2mm RTLBE , Moro+ , Tonic neck+ ,Sucking and rooting +

  • Problem lists

    1. History of Vaccination (DPT-HB 4 days PTA)2. Generalized Petechial hemorrhage (2 days PTA)3. Nausea and vomiting 2 days PTA4. Thrombocytopenia with Lymphocyte predominate

    with anemia

  • Provisional diagnosis- ITP

    DDx

    - Microangiopathic Hemolytic anemia (MAHA)- Von Willebrand disease - Collagen vascular disease- Vitamin C def.

  • Investigation

    CBC ( 25/07/57)Hb 9 g/dl Hct 26.6 %

    Hypochromia 1+ Anisocytosis few Microcytosis few

    platelet count 8,000/mm3 ,platelet smear : decrease

    WBC 7,920 /mm3

    PMN 15%, L 77 %, M 2%, E 2%, B 0%, Blast cell 4

    MCV 75.1 fl, MCH 25.4 pg

  • Chest X-ray

    Normal bony structure, No pleural effusion, No lung infiltration, No cardiomegaly

  • Impression :Immune thrombocytopenic purpura

  • ManagementAdmitSpecific treatment Hydrocortisone

    30 mg. iv. q 6 hr. CBC tomorrow

    Supportive treatment Observe clinical (Observe bleeding and neuro sign) Regular diet

  • 26/7/57

    S:

    O: BT 36.5 PR 136/min full RR 36/minAbdomen : soft, not tenderExt: generalized petechiaeA: ITP

    Clinical and vital sign stable , no active bleedingP: Cont. hydrocortisone

  • CBC ( 26/07/57)Hb 9 g/dl Hct 27.4 %

    Hypochromia 1+ Anisocytosis few Microcytosis

    few Polychromasia 1+

    platelet count 71,000/mm3 ,platelet smear :

    decrease

    WBC 11,500 /mm3

    PMN 61%, L37 %, M 2%, E0 %, B 0%

    MCV 75.1 fl, MCH 24.7 pg

  • 27/7/57S:

    O: BT 36.5 PR 140/min full RR 36/minAbd: Normoactive bowel sound, distend abdomen, soft, not tenderExt: generalized petechiae

    A: ITPClinical and vital sign stable , no active bleeding

    P: CBC , if platelet 80,000 D/C F/U 2 weeks with CBC

  • CBC ( 27/07/57)Hb 9.4 g/dl Hct 29 %

    Spherocyte 1+ Schistocyte few Polychromasia 1+

    platelet count 262,000/mm3

    WBC 11,700 /mm3

    PMN 57%, L40 %, M 3%, E0 %, B 0%

    MCV 74.8 fl, MCH 24.7 pg

  • Discharge 27/7/57 F/U 2weeks with CBC

    Home medicationPrednisolone (1MKD) X 20 tabs

    1x1 po pcSimethicone x I

    0.3 ml po tid ac

  • Hemostasis

  • Primary hemostasis

    Platelet adhesion platelet exposed subendothelial

    layerPlatelet activation

    resting activated platelet

    Platelet aggregation platelet

  • Secondary hemostasis

  • Tertiary hemostasis

  • Approach to bleeding disorder

    Local or Systemic bleeding Primary or Secondary Hemostatic defect Congenital or acquired Initial investigation Diagnosis and treatment

  • 1. Local or Systemic bleeding (local bleeding)

    (systemic bleeding) systemic bleeding

    (massive bleeding) (prolong bleeding) (unrelated to injury) (multiple site bleeding) (spontaneous bleeding) (delay bleeding) (uncommon site bleeding)

    (hemathrosis) (intracerebral hemorrhage)

  • 2.Primary or Secondary Hemostatic defect

    Platelet Coagulation disorders factor disorders

    Site of bleeding Skin Deep in soft tissuesMucous membranes (joints, muscles)

    (epistaxis, gum,vaginal, GI tract)

    Petechiae Yes No Ecchymoses (bruises) Small, superficial Large, deep Hemarthrosis / muscle bleeding Extremely rare Common Bleeding after cuts & scratches Yes No Bleeding after surgery or trauma Immediate, Delayed (1-2 days),

    usually mild often severe27

  • 3.Congenital or acquired ( ) : Aspirin :

    Hemophilia A, B >>> X-linked recessive

    Hemophilia C >>> Autosomal recessiveVon Willebrand disease >>> Autosomal dominantBernard Soulier >>> Autosomal recessive Glanzmann thrombasthenia >>> Autosomal recessive

  • Initial investigation primary hemostasis (thrombocytopenia)

    (platelet dysfunction) CBC peripheral blood smear

    thrombocytopenia

    bleeding time platelet aggregation test

  • secondary hemostasis coagulogram PT aPTT

    CBC, platelet aggregation test, PT aPTT

    Mild bleeding disorders mild von Willebranddisease (vWD)

    Factor XIII deficiency Hyperfibrinolysis Vascular disease Ehler Danlos syndrome,

    Osler Weber Rendu syndrome, Marfansyndrome, Scurvy

    Initial investigation (cont.)

  • Approach to thrombocytopenia

    aplasia infiltration ineffective megakaryopoiesis

    eg. MDS selective impairment of platelet production

    Causes of splenomegaly infection inflammation congestion maligancy red cell disorders storage diseases

    immuneauto-immune (ITP, SLEdrugsinfectionsallo-immune

    non-immunesepsisDIC, TTP, HUShypertensive disorders of pregnancy

    look for splenomegaly

    bone marrow investigationreview meds

    look for underlying disordersreview meds

    THROMBOCYTOPENIA

    rule out pseudothrombocytopenia

    SEQUESTRATION PRODUCTION DESTRUCTION

  • Immune thrombocytopenic

    purpura (ITP)

  • ITP

    An autoimmune Ab affecting platelets. Platelet counts 100,000 cell/mm3

    Newly diagnosed ITP Persistent ITP Chronic ITP Refractory ITP

    34

  • ITP - EtiologyMost common occur in the young

    70% 1-10 yrs. 20 % 10-16 yrs. 10 % 3-12 months

    Most pediatric cases appear to be related to

    sensitization by viral infection 1-4 wk before the onset

    of thrombocytopenia .

    Infections include a non-specific URI, Rubella,

    Varicella, Measles , Pertussis, Mumps, EBV, CMV,

    HIV and bacteria or MMR vaccine

  • ITP - PATHOGENESIS

    Infection >>> IgG antibodies directed against specific platelet antigens or antigen-antibody complexes (viral antigen-antiviral antibody complex on the platelet surface)

    Platelet Ab Macrophage Fc receptors

    Platelet Ab to megakaryocyte

    36

  • ITP: Clinical features The classic presentation of ITP is a previously healthy

    1-4 yr old

    child who has sudden onset of generalized petechiae

    and purpura.

    typically in children and young female

    viral infection

    37

  • signs and symptoms (insidious onset)

    : superficial ecchymosis , petechiae.

    Epistaxis 25 % of case but not severe.

    Hepatosplenomegaly was uncommon.

    Severity depend on platelet count.

    ICH 0.1-0.5 %

  • ITP

    ClinicalMucocutaneous bleeding

    100%Epistaxis 25%GI bleeding 2-8%

    Hematuria 1-10%CNS bleeding 0-3%

    39

  • Diagnosis

    Age : 1-10 yrsAcute onset of bleeding No feverNo hepatosplenomegalyNo lymphadenopathyCBC : Platelet count < 100,000 / mm3

  • ITP

    Investigation ITP IS A DIAGNOSIS OF EXCLUSION CBC : Low plt. Count ( blood smear : giant plt.) Coagulogram (PT, aPTT, TCT) : normal Bleeding time : prolong (plt

  • ITP

    42

  • Management

    Restrict activityAvoid aspirin, non-steroidal anti-inflammatory drugs, antihistamines

    Observe clinical 6-12

    43

  • 2011 Clinical Practice Guideline on the Evaluation and

    Management of Immune Thrombocytopenia (ITP)

  • Initial Management of ITP1. Assessment of Disease Status: What bleeding is the patient experiencing?

    Determine the timing, location, and severity of

    bleeding symptoms. Does this patient have any additional risk factors for bleeding such as use of antithrombotic agents orhigh-risk occupation? Is a surgical procedure anticipated?

    Is this patient likely to comply with recommended treatments?

    Is the bleeding experienced by this patient interfering with his or her daily activities or causing significant anxiety?

  • 2.General Considerations for Initial Management: The majority of patients with no bleeding or mild bleeding (defined here as skin manifestations only, such as petechiae and bruising) can be treated with observation alone regardless of platelet count. First-line treatment includes observation, corticosteroids, IVIg, or anti-D immunoglobul