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    DR - Tjatur Winarsanto SPPD RST Ciremai

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    iqbalyanuarr
    1) Idiopathic thrombocytopenic purpura (ITP) is characterized by low platelet counts without an identifiable cause and normal white and red blood cell counts. 2) ITP has an annual incidence of 22 million cases worldwide and is more prevalent and often chronic in adults. Clinical manifestations include bruising, bleeding, and in rare cases bleeding in the brain or gastrointestinal tract. 3) The goal of ITP treatment is to raise platelet counts to a safe level to prevent bleeding, not necessarily cure the underlying condition, with options including corticosteroids, IV immunoglobulin, anti-D immunoglobulin, and splenectomy.

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    DR - Tjatur Winarsanto SPPD RST Ciremai

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    iqbalyanuarr
    43 views29 pages
    1) Idiopathic thrombocytopenic purpura (ITP) is characterized by low platelet counts without an identifiable cause and normal white and red blood cell counts. 2) ITP has an annual incidence of 22 million cases worldwide and is more prevalent and often chronic in adults. Clinical manifestations include bruising, bleeding, and in rare cases bleeding in the brain or gastrointestinal tract. 3) The goal of ITP treatment is to raise platelet counts to a safe level to prevent bleeding, not necessarily cure the underlying condition, with options including corticosteroids, IV immunoglobulin, anti-D immunoglobulin, and splenectomy.

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    Itp

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    1) Idiopathic thrombocytopenic purpura (ITP) is characterized by low platelet counts without an identifiable cause and normal white and red blood cell counts. 2) ITP has an annual incidence of 22 million cases worldwide and is more prevalent and often chronic in adults. Clinical manifestations include bruising, bleeding, and in rare cases bleeding in the brain or gastrointestinal tract. 3) The goal of ITP treatment is to raise platelet counts to a safe level to prevent bleeding, not necessarily cure the underlying condition, with options including corticosteroids, IV immunoglobulin, anti-D immunoglobulin, and splenectomy.

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    43 views29 pages

    DR - Tjatur Winarsanto SPPD RST Ciremai

    Uploaded by

    iqbalyanuarr
    1) Idiopathic thrombocytopenic purpura (ITP) is characterized by low platelet counts without an identifiable cause and normal white and red blood cell counts. 2) ITP has an annual incidence of 22 million cases worldwide and is more prevalent and often chronic in adults. Clinical manifestations include bruising, bleeding, and in rare cases bleeding in the brain or gastrointestinal tract. 3) The goal of ITP treatment is to raise platelet counts to a safe level to prevent bleeding, not necessarily cure the underlying condition, with options including corticosteroids, IV immunoglobulin, anti-D immunoglobulin, and splenectomy.

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    ITP

    Dr.Tjatur Winarsanto SpPD


    RST Ciremai
    Idiopathic (Immune)
    Thrombocytopenic Purpura

     Thrombocytopenia in the absence of other


    blood cell abnormalities (normal RBC &
    WBC, normal peripheral smear)

     No clinically apparent conditions or


    medications that can account for
    thrombocytopenia
    Statistics of ITP
     Incidence of 22 million/year in one study

     Prevalence greater as often chronic


    *Segal et al 100 million/year
    *age-adjusted prevalence 9.5/100,000
    *1.9 :1 females / males
    Clinical Manifestations
     May be acute or insidious onset
     Mucocutaneous Bleeding
    *petechiae, purpura, ecchymosis
    *epistaxis, gum bleeding
    *menorrhagia
    *GI bleed, CNS bleed = RARE
    Etiology of ITP : Children
     Often after infection (viral or bacterial)

     Theories:
    *antibody cross-reactivity
    *H. pylori
    *bacterial lipopolysaccharides
    Etiology of ITP : Adults

     ?? Auto-antibodies?
    Diagnosis (of Exclusion)
     Rule out other causes:
    *lab error / PLT clumping
    *drug / medication interaction
    *infections (HIV, Hepatitis C)
    *thyroid / autoimmune disease
    *destructive / consumptive processes (TTP/HUS)
    *bone marrow disease (leukemias, MDS)
    Diagnosis (of Exclusion)
     Rule out other causes:
    *lab error / PLT clumping
    *drug / medication interaction
    *infections (HIV, Hepatitis C)
    *thyroid / autoimmune disease
    *destructive / consumptive processes (TTP/HUS)
    *bone marrow disease (leukemias, MDS)
    To Marrow or Not to Marrow?
     Bone marrow aspiration & biopsy if…

     Patient 60 yrs. or older


     Poorly responsive to tx
     Unclear clinical picture
    Anti-Platelet Antibody Testing
     NOT recommended by ASH Practice
    Guidelines
     Poor positive/negative predictive values,
    poor sensitivity with all current testing
    methods…
     …and doesn’t change the management!
    Management of ITP in Adults
     Emergency vs. Chronic Tx

     Goal = prevention of bleeding, NOT cure!


    Management of ITP in Adults
     Emergency vs. Chronic Tx

     Goal = prevention of bleeding, NOT cure!


    General Principles of Therapy
     Major bleeding rare if PLT > 10,000

     Goal = get PLT count to safe level to


    prevent bleeding…

     …not to specifically cure the ITP!


    “Safe” Platelet Counts
     “moderately” t-penic = 30-50,000

     Probably safe if asymptomatic

     Caution with elderly (CNS bleeds)


    When Planning Therapy…
     Tailor therapy and decision to treat to the
    individual patient

     Weigh bleeding vs. therapy risks


    Initial Therapy
     Prednisone 1 mg/kg/day
    *usually response within 2 weeks

     Taper off after PLT response

     Duration of use = controversial


    Second-Line Therapy
     IV Immune Globulin (IVIg)
    1 gram/kg/day x 2 days

     WinRho (anti-D) – if pt is Rh+


    50-75 mcg/kg/day
    Treatment Side-Effects
     Steroids
    *bone density loss *GI effects
    *muscle weakness *weight gain

     IVIG/anti-D
    *hypersensitivity *headache
    *renal failure *nausea/vomiting
    *alloimmune hemolysis
    Splenectomy
     Usually reserved for treatment failure

     Consider risk of bleeding, pt lifestyle

     RISKS
    *surgical procedure
    *loss of immune function  vaccinations
    When to do Splenectomy?

    Data from George, JN, Woolf, SH, Raskob, GE, et al. Blood 1996; 88:3.
    Response Post-Splenectomy
     Usually normalized PLTs within 2 weeks
    (often immediately)

     Younger pts do better

     Kojouri et al (Blood 2004)  65% CR


    Data from Fabris, F, et al. Br J Haematol 2001; 112:637.
    Chronic Refractory ITP
     Persistent > 3 months
     PLT < 50,000
     Failure to respond to splenectomy
    When all else fails…
     Steroids
     IVIg / anti-D
     Rituximab (anti-CD20)
     Cyclophosphamide
     Danazol
     Accessory splenectomy
     H. pylori eradication
    Wrapping it up…
     ITP is often a chronic disease in adults
     Multiple therapies may be needed over
    time
     Goal = prevention of complications
     Therapy needs to be tailored to the
    individual patient

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