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The Diagnosis and Management of Chronic Immune Thrombocytopenia in Adults

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The Diagnosis and Management of Chronic Immune Thrombocytopenia in Adults Immune thrombocytopenic purpura The diagnosis and management of chronic immune thrombocytopenia in adults A. Newland ABSTRACT Barts and the London School Primary immune thrombocytopenia (ITP) is a complex autoimmune disorder involving antibody- and cell-mediated destruction of platelets and suppression of platelet production. The definition of ITP of Medicine and Dentistry, 9 Queen Mary University of London, requires an isolated thrombocytopenia with a platelet count below 100×10 /L, and with no other obvi- London, United Kingdom ous cause or clinically evident secondary form of immune thrombocytopenia. There is no “gold stan- dard” test for ITP, but the diagnosis is based on exclusion. ITP is a minor disease for many patients; however, the natural history is variable and unpredictable. Many patients who are in otherwise good health can be left untreated, especially if their platelet Hematology Education: 9 the education program for the counts are close to or above 20×10 /L. The overall mortality rate for ITP is less than 1%, and the mor- annual congress of the European bidity and mortality associated with treatment can be worse than the disease. Hematology Association While there is general agreement over treatment of the newly presenting adult with ITP, few evi- dence-based studies direct therapy in the relapsed and refractory patient. There is an understanding 2011;5:184-190 that the patient should be treated for their clinical state rather than their platelet count but not what second line treatment should be used and in what order. The options will be considered in this review. disease duration, the term “newly diagnosed Introduction ITP” has been adopted for all cases at diag- Primary immune thrombocytopenia (ITP) nosis. A new category, called “persistent is an autoimmune disorder typified by a ITP,” was introduced for patients with ITP to platelet count below the normal range. define the period lasting between 3 and 12 Clinical manifestations are related to the months from diagnosis. This category severity of thrombocytopenia and include includes patients not achieving spontaneous purpura and bleeding episodes, which can be remission or not maintaining their response potentially life-threatening and require after stopping treatment between 3 and 12 emergency treatment. In the past, “acute months from diagnosis. The term “chronic ITP” has been used to describe a self-limited ITP” is used for patients with ITP lasting for form of the disease (e.g., secondary to viral more than 12 months. illness) and “chronic ITP” identified ITP last- Acute abrupt-onset ITP, with duration of ing for more than 6 months. This definition less than 6 months, occurs mainly in chil- was felt to be unsatisfactory, and a more dren, often following viral infection or coherent staging scheme was felt necessary immunization, with boys and girls being in order to compare studies and define the equally affected. Chronic ITP is typically disease. seen in adults, more often affecting women The terminology was agreed and defini- of childbearing age. In Europe, the incidence tions published in 2009 by Rodeghiero et al. of ITP in adults has been estimated to be on behalf of an International Working between 1 and 4 per 100,000 persons.2–4 Group.1 The abbreviation ITP now stands for When a cut-off platelet count of less than 50 immune thrombocytopenia and not immune x 109/L was applied, the annual incidence of thrombocytopenic purpura. It is defined as a ITP was estimated to be 2.25 per 100,000 in platelet count of under 100×109/L without Denmark2 and 1.6 per 100,000 in the UK.4 any other cause of thrombocytopenia. A In up to 30% of patients with immune new staging scheme was also proposed, thrombocytopenia, the cause may be sec- which defines the following stages of ITP: ondary to infection (HIV, HCV, CMV, or • Newly diagnosed ITP: months 0–3 after EBV), drugs, another autoimmune disorder presentation (e.g., SLE) or in association with a malignan- • Persistent ITP: months 4–12 cy (malignant lymphoma or chronic lympho- • Chronic ITP: greater than 12 months cytic leukemia; CLL). • Refractory ITP: patient has failed splenec- The natural history of ITP is variable and tomy unpredictable. For patients who are in other- • Severe ITP: patient has had major clinical wise good health, many can be left untreat- bleeding. ed, especially if their platelet counts are close In the absence of reliable predictive clinical to or above 20×109/L. The overall mortality or laboratory tests, variables parameters of rate for ITP is less than 1%, and the morbid- | 184 | Hematology Education: the education programme for the annual congress of the European Hematology Association | 2011; 5(1) London, United Kingdom, June 9-12, 2011 ity and mortality associated with treatment – often Hodgkin lymphoma), liver disease, alcohol abuse, med- related to infections – can be worse than the disease. ications (prescription or non-prescription), environmen- Thus, platelet count and risk for bleeding events should tal toxins, quinine exposure, primary bone marrow dis- be considered before treatment of ITP is initiated. A eases, and recent transfusions or immunizations should guideline describing the consensus of management in all be considered and where possible, excluded. Europe was published in 2003.5 These guidelines fol- There is much controversy over the incidence and lowed those of the American Society of Hematology in importance of immunizations, which are infrequently 1996.6 It was generally felt that these needed updating but clearly associated with thrombocytopenia. Measles, due, in part, to the introduction of new classes of thera- mumps, rubella (MMR) is typical, and it is estimated peutic agents and a greater understanding of the disease that one in 40,000 children given MMR will develop pathophysiology. The International Consensus Report severe thrombocytopenia about 1–2 weeks post vacci- on the diagnosis and treatment of ITP were published in nation. It is associated with platelet-reactive anti-glyco- 2010.7 The writing committee reviewed over 1000 man- protein antibodies, and is self limited in 80% of uscripts written in the last 20 years to try to establish patients. In the remaining 20%, thrombocytopenia per- working guidelines, evidence based where possible, that sists but is responsive to ITP specific therapy, and recur- could subsequently be used, in conjunction with the rence is rare.9 new terminology, to review practice and provide future Inherited thrombocytopenias should be considered in audited treatment advice. ASH have subsequently patients who have a life-long history of thrombocytope- updated their guidelines with broadly similar recom- nia or suggestive family history. mendations. The main difference is the recommenda- tion that splenectomy remains the main second line treatment of chronic ITP, which in the light of newer Anti-platelet and other auto-antibodies options and patient preference, is increasingly contro- and their role in the pathogenesis of ITP versial.8 In early 1980 it was noted that sera or eluates of platelets from patients with ITP would bind to normal Diagnosis platelets but only about one-quarter would bind to the platelets of patients with Glanzmann’s thrombasthe- There is no “gold standard” test for ITP, but the diag- nia.10 It was speculated that ITP patients produced auto- nosis is one based on exclusion of other causes of isolat- antibodies against either platelet glycoprotein (GP) IIb ed thrombocytopenia. The essential investigations or GPIIIa since thrombasthenic patients lack these pro- include history and physical examination, full blood teins. Since that time, several laboratories have provided count, and examination of the blood smear. A bone direct evidence for the presence of auto-antibodies marrow examination is now considered to be necessary against GPIIb/IIIA and other platelet antigens in ITP.11–14 in only a few selected groups of patients: those over 60 In the meantime, platelet kinetic studies using radio- years of age, those who fail to respond to therapy or labeling methods had reignited the debate about the have relapsed after therapy, or non-responding patients mechanisms of thrombocytopenia in ITP. These studies being considered for splenectomy, and some of these showed shortened platelet survival, and high rates of remain debatable. All adult patients should be screened platelet production ranging from 4 to 9 times normal in for HIV and hepatitis C, and in appropriate countries, patients with ITP.15–17 With the introduction of 111In, the for H. pylori. small numbers of autologous platelets in ITP could be Some tests should be considered in special circum- adequately labeled, and it became evident that there stances. In patients who might go on to receive intra- was considerable heterogeneity in platelet turnover venous immunoglobulin, an underlying immunoglobu- between patients, with a substantial proportion having lin deficiency should be considered and the IgG level platelet production within normal limits even though checked. This is particularly important in children to the mean cell life of platelets was significantly reduced exclude Common Variable Immune deficiency, but compared with that in healthy subjects.18–20 If platelet should also be considered in adults. In patients being destruction were the only mechanism to cause throm- considered for anti-RhD, the Rh group and direct bocytopenia, then platelet production would be expect- antiglobulin test (DAT) should also be evaluated. ed to increase to offset low platelet counts. It was, Hepatitis B should also be screened for in those who therefore, proposed once again that thrombocytopenia may receive the anti-CD20 monoclonal antibody ritux- may result not only from platelet destruction, but also imab. from antibody-mediated damage to megakaryocytes. Testing for glycoprotein-specific antibodies may con- Evidence to support this hypothesis has accumulated firm the diagnosis but the tests lack sensitivity and are over time. rarely used routinely. Testing for anti-phospholipid anti- The infections most closely linked with ITP are HIV, bodies and other auto-antibodies is not useful diagnos- HCV, and H.
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