Idiopathic thrombocytopenic purpura – pathogenesis, modern therapy, and an analysis of its prevalence, diagnostics and therapy in the South Moravian Region
Authors:
L. Červinek; M. Doubek; J. Mayer
Authors‘ workplace:
– pro život (CELL , The CzEch Leukemia Study Group – for Life)
; Interní hematoonkologická klinika LF MU a FN Brno, kooperující pracoviště České leukemické skupiny
Published in:
Transfuze Hematol. dnes,15, 2009, No. 1, p. 23-30.
Category:
Comprehensive Reports, Original Papers, Case Reports
Overview
Idiopathic thrombocytopenic purpura (ITP) is ranged among autoimmune diseases. ITP is defined by isolated thrombocytopenia. ITP is usually diagnosed per exclusionem. Pathogenesis of ITP is complex and implicates interaction of T and B lymphocytes, antigen presenting cells and organs of the monocyto-macrophage system. In the pathogenesis of ITP, affliction of megakaryocytes is invoked as well. Therapy of ITP is recommended when the platelet count drops to 20–30 x 109/l and should be individualized according to the risk profile of the patient. As the first-line treatment corticoids, immunoglobulins (IVIG) and anti-D immunoglobulin are used. The second-line treatment is splenectomy and rituximab. The third-line treatment is immunosuppressive and immunomodulant therapy. Therapy using thrombopoetin agonists represents promise for patients with chronic refractory form of ITP. In the article we also discuss diagnostic and therapeutic preferences of hematologists in different work sites in the South Moravian Region. According to our analysis the incidence of ITP is 6 cases of 100 000 inhabitants, and its prevalence is 50.3 cases of 100 000 inhabitants.
Key words:
ITP, pathogenesis, megakaryocytes, thrombopoietin, thrombopoietin agonists
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Transfusion and Haematology Today
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