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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