Management of adult idiopathic thrombocytopenic purpura

Department of Pathology, Hospital of the University of Pennsylvania, Philadelphia, Pennsylvania 19104, USA.
Annual Review of Medicine (Impact Factor: 15.48). 02/2005; 56:425-42. DOI: 10.1146/
Source: PubMed

ABSTRACT Idiopathic thrombocytopenic purpura (ITP) is a common hematologic disorder manifested by immune-mediated thrombocytopenia. The diagnosis remains one of exclusion, after other thrombocytopenic disorders are ruled out based on history, physical examination, and laboratory evaluation. The goal of treatment is to raise the platelet count into a hemostatically safe range. The disorder is usually chronic, although there is considerable variation in the clinical course and most patients eventually attain safe platelet counts off treatment. However, a subset of patients has severe disease refractory to all treatment modalities, which is associated with considerable morbidity and mortality. This article focuses on the management of primary ITP in adults. We discuss criteria for treatment, the roles of splenectomy and other treatment options along with their side effects, and the management of ITP during pregnancy.

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    • "The established treatment practices for chronic ITP have also undergone a major change. After a long period of standard treatment approach using steroids, splenectomy, and immunosuppressive therapy, a breakthrough came with the use of B-cell depletion therapy (Cines and McMillan, 2005). The availability of monoclonal antibody, rituximab, has led hematologists to reconsider the role of splenectomy in the frontline management of chronic ITP (Arnold et al., 2007). "
    Autoimmune Disorders - Current Concepts and Advances from Bedside to Mechanistic Insights, 11/2011; , ISBN: 978-953-307-653-9
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    • "Clinical response of ITP patients to therapy that inhibits phagocytosis The importance of platelet destruction in ITP is further supported by the demonstration that splenectomy results in a complete, unmaintained remission in about two-thirds of ITP patients (Branehog, 1975; Gernsheimer et al, 1989; Louwes et al, 2001). In addition, agents such as intravenous IgG, anti- D and corticosteroids, which work, in part, by interfering with phagocytosis, may result in a temporary normalization of the platelet count (Cines & McMillan, 2005). "
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    ABSTRACT: Chronic immune thrombocytopenia (ITP) is a haematological disorder in which patients predominantly develop skin and mucosal bleeding. Early studies suggested ITP was primarily due to immune-mediated peripheral platelet destruction. However, increasing evidence indicates that an additional component of this disorder is immune-mediated decreased platelet production that cannot keep pace with platelet destruction. Evidence for increased platelet destruction is thrombocytopenia following ITP plasma infusions in normal subjects, in vitro platelet phagocytosis, and decreased platelet survivals in ITP patients that respond to therapies that prevent in vivo platelet phagocytosis; e.g., intravenous immunoglobulin G, anti-D, corticosteroids, and splenectomy. The cause of platelet destruction in most ITP patients appears to be autoantibody-mediated. However, cytotoxic T lymphocyte-mediated platelet (and possibly megakaryocyte) lysis, may also be important. Studies supporting suppressed platelet production include: reduced platelet turnover in over 80% of ITP patients, morphological evidence of megakaryocyte damage, autoantibody-induced suppression of in vitro megakaryocytopoiesis, and increased platelet counts in most ITP patients following treatment with thrombopoietin receptor agonists. This review summarizes data that indicates that the pathogenesis of chronic ITP may be due to both immune-mediated platelet destruction and/or suppressed platelet production. The relative importance of these two mechanisms undoubtedly varies among patients.
    British Journal of Haematology 06/2009; 146(6):585-96. DOI:10.1111/j.1365-2141.2009.07717.x · 4.96 Impact Factor
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    ABSTRACT: Autoimmune thrombocytopenic purpura is the most common autoimmune disorder encountered in the pregnant patient. It is potentially fatal for the mother and fetus yet treatable and potentially curable. Analysis of current perinatal literature reveals not only a great deal of interest and activity in the study of this syndrome and its special problems during pregnancy but also significant controversy. The disease can be acute or chronic and vary in time of onset and severity of manifestations. If not forewarned with an awareness of this disorder's pathogenesis and potential fetal effects particularly in the pregnant woman who has undergone splenectomy, the obstetrician cannot respond appropriately. The usefulness of platelet antibody determinations to facilitate obstetric management decisions is discussed. The importance of cooperative care among the obstetrician, hematologist, and neonatologist is emphasized. Recommendations for management of autoimmune thrombocytopenic purpura in pregnancy are derived from a review of current concepts of the disorder's pathogenesis, pathophysiology, criteria for diagnosis, and modes of therapy as well as special maternal/fetal considerations of antepartum, intrapartum, and postpartum care.
    American Journal of Obstetrics and Gynecology 10/1984; 150(1):86-96. DOI:10.1016/S0002-9378(84)80115-5 · 3.97 Impact Factor
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