immune thrombocytopenia
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IMMUNE THROMBOCYTOPENIA

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IMMUNE THROMBOCYTOPENIA. Cathy Payne MSN, ACNP-BC Hematology/Oncology Nurse Practitioner Ironwood Cancer and Research Centers. OBJECTIVES. • Define ITP • Terminology • Epidemiology • Mortality/Morbidity • Pathophysiology • Diagnosis • ASH guidelines/Management.

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immune thrombocytopenia

IMMUNE THROMBOCYTOPENIA

Cathy Payne MSN, ACNP-BC

Hematology/Oncology Nurse Practitioner

Ironwood Cancer and Research Centers

objectives
OBJECTIVES

• Define ITP

• Terminology

• Epidemiology

• Mortality/Morbidity

• Pathophysiology

• Diagnosis

• ASH guidelines/Management

immune thrombocytopenia1
Immune Thrombocytopenia
  • ITP is an autoimmune-mediated hematologic disorder, in which the patients immune system produces antibodies directed against platelet antigens resulting in immune mediated platelet destruction and/or suppression of platelet production
  • Cines, D.B., Blanchet, VS, (2002) Immune thrombocytopenia purpura.
pathophysiology
PATHOPHYSIOLOGY
  • reduced platelet lifespan due to antibody mediated destruction, as well as impaired platelet production
  • specific IgG autoantibodies produced by the patient’s B cells most often are directed against
  • platelet membrane glycoproteins like GPIIb/IIIa inciting events- viral infections and systemic conditions, malignancies
terminology
TERMINOLOGY
  • Diagnostic platelet count <100,000
  • Primary vs secondary
  • Duration
  • Severity
  • Refractory ITP
  • Response to treatment
epidemiology
EPIDEMIOLOGY
  • 1 to 3 per 100,000 adults
  • Young women
  • Incidence increases with age with a slight male predominance
morbidity mortality
MORBIDITY/MORTALITY
  • Most patients will have good outcomes with infrequent hospital admissions and no excess mortality compared with the general population
  • patients with persistent severe thrombocytopenia not responding to therapy within first 2 years have considerable M/M
  • Bleeding/infections cause of death
diagnosing itp
DIAGNOSING ITP
  • HISTORY
    • ethanol history
    • liver disease
    • drug history
    • bleeding/bruising
    • family history
    • risk factors for HIV or hepatitis C and or B symptoms
    • Symptoms of MDS
    • autoimmune disorders
    • recent viral infection
    • history of transfusions
physical exam
PHYSICAL EXAM
  • petechiae
  • purpura
  • conjunctival bleeding
  • retinal hemorrhages
  • lesions on mucosal membranes
  • enlarged liver or spleen
  • lymph nodes
  • sighs of sepsis, DIC or other severe systemic abnormalities
  • pregnancy
complete blood count
COMPLETE BLOOD COUNT
  • Isolated thrombocytopenia, plt count < 100,000
  • anemia only if significant bleeding otherwise normal red cell indices, WBC count and differential, normal coagulation parameters
  • peripheral blood smear- schistocytes
lab tests
LAB TESTS

• HIV and Hep C

• coagulation studies

• Helicobacter pylori

• thyroid funtion

• rheumatological studies

• Vitamin B12 and folate levels

• anti-platelet antibody testing- NO

bone marrow exam
BONE MARROW EXAM
  • not always needed, but do if the etiology of thrombocytopenia is unclear
  • any abnormality with History/Physical, blood smear or blood indices
  • yes for those older than 60 years
differential diagnosis
DIFFERENTIAL DIAGNOSIS

• drug induced

• infections

• liver disease and hypersplenism

• microangiopathic processes

• MDS and other bone marrow disorders

• vasculiticpurpura

ash guidelines management of itp initial management
ASH GUIDELINES/MANAGEMENT OF ITP INITIAL MANAGEMENT
  • consider treatment for patients with a platelet count <30,000
  • longer courses of corticosteroids are preferred over shorter courses of corticosteriods or IVIG
  • IVIG may be used in conjunction with corticosteriods if a more rapid increase in platelet count is required
  • either IVIG (1g.kg for one dose, repeated as necessary) or anti-D (in appropriate patients) may be used as a first line treatment if corticosteriods are contraindicated
ash guidelines for sencond line therapy
ASH GUIDELINES FOR SENCOND LINE THERAPY

• splenectomy

• rituximab

• thrombopoientin receptor agonist

• high dose dexamethasone

• immunosuppression

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