Pathophysiology, Diagnosis and Treatment of Immune Thrombocytopenia

Authors

DOI:

https://doi.org/10.5195/ijms.2017.171

Keywords:

primary immune thrombocytopenia, ITP, guidelines, thrombopoietin receptor agonists, splenectomy, immune thrombocytopenic purpura

Abstract

Immune thrombocytopenia (ITP) is an acquired disorder characterized by isolated thrombocytopenia with a peripheral blood count < 100.000/ mm3 in the absence of any obvious initiating or underlying causes, by antibody mediated destruction of platelets and suppression of megakaryocyte and platelet production on the basis of immune deregulation. ITP is idiopathic (primary) in 80% of cases and secondary to several associated disorders in 20% of cases. A diagnosis of exclusion, based on patient history, physical examination, complete blood count and examination of the peripheral blood smear, is used for ITP. The treatment of ITP is indicated in adult patients with platelet counts below 20.000-30.000/ mm3, with bleedings or risk for bleeding. First line therapy is represented by corticosteroids, intravenous immunoglobulins and intravenous anti-RhD. Second-line treatment is represented by: splenectomy, inhibition of the monocytic phagocytic system therapy, immunosupressive therapy, anabolic steroids, anti-CD20 therapy, and thrombopoietin receptor agonists.

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Author Biography

Mihnea-Alexandru Găman, Carol Davila” University of Medicine and Pharmacy, Bucharest, Romania.

Mihnea- Alexandru Găman is currently a 4th year medical student of ”Carol Davila”University of Medicine and Pharmacy, Bucharest, Romania of a six year program. He is currently working on a research project concerning the molecular analysis of the Factor VIII (F8) gene, funded by the Society of Students in Medicine of Bucharest (SSMB).

References

1. Rodeghiero F, Stasi R, Gernsheimer T, Michel M, Provan D, Arnold DM, et al. Standardization of terminology, definitions and outcome criteria in immune thrombocytopenic purpura of adults and children: report from an international working group. Blood. 2009;113(11):2386-93.
2. Provan D, Stasi R, Newland AC, Blanchette VS, Bolton-Maggs P, Bussel JB, et al. International consensus report on the investigation and management of primary immune thrombocytopenia. Blood. 2010;115(2):168-86.
3. Johnsen J. Pathogenesis in immune thrombocytopenia: new insights. Hematology Am Soc Hematol Educ Program. 2012;2012:306-12.
4. Stasi R. Immune pathophysiology of primary immune thrombocytopenia. Hematology Education: the education program for the annual congress of the European Hematology Association. 2011;5:173-8.
5. Kuwana M, Kaburaki J, Okazaki Y, Miyazaki H, Ikeda Y. Two types of autoantibody- mediated thrombocytopenia in patients with systemic lupus erythematosus. Rheumatology (Oxford). 2006;45(7):851-4.
6. Moulis G, Audemard-Verger A, Arnaud L, Luxembourger C, Montastruc F, Gaman AM, et al. Risk of thrombosis in patients with primary immune thrombocytopenia and antiphospholipid antibodies: A systematic review and meta-analysis. Autoimmun Rev. 2016;15(3):203-9.
7. Gaman A, Gaman M. The evaluation of oxidative stress in chronic idiopathic thrombocytopenic purpura (ITP). Haematologica. 2015;100:772-772.
8. Zhang B, Zehnder JL. Oxidative stress and immune thrombocytopenia. Semin Hematol. 2013;50(3):e1-4.
9. Gaman A, Gaman M. The antioxidant treatment in patients with immune thrombocytopenia. Haematologica. 2014;99:773-.773
10. George JN, Woolf SH, Raskob GE, Wasser JS, Aledort LM, Ballem PJ, et al. Idiopathic thrombocytopenic purpura: a practice guideline developed by explicit methods for the American Society of Hematology. Blood. 1996;88(1):3- 40.
11. Zen M, Canova M, Campana C, Bettio S, Nalotto L, Rampudda M, et al. The kaleidoscope of glucorticoid effects on immune system. Autoimmun Rev. 2011;10(6):305-10.
12. Newland AC, Treleaven JG, Minchinton RM, Waters AH. High-dose intravenous IgG in adults with autoimmune thrombocytopenia. Lancet. 1983;1(8316):84-7.
13. Kaveri S. Intravenous immunoglobulin: Exploiting the potential of natural antibodies. Autoimmunity Reviews. 2012;11(11):792-4.
14. Despotovic J, Lambert M, Herman J, Gernsheimer T, McCrae K, Tarantino M, et al. RhIG for the treatment of immune thrombocytopenia: consensus and controversy (CME). Transfusion. 2012;52(5):1126-36.
15. Barsam S, Psaila B, Forestier M, Page L, Sloane P, Geyer J, et al. Platelet production and platelet destruction: assessing mechanisms of treatment effect in immune thrombocytopenia. Blood. 2011;117(21):5723-32.
16. Liebman H, Pullarkat V. Diagnosis and Management of Immune Thrombocytopenia in the Era of Thrombopoietin Mimetics. Hematology-American Society Hematology Education Program. 2011:384-90.
17. Cines D, Bussel J, Liebman H, Prak E. The ITP syndrome: pathogenic and clinical diversity. Blood. 2009;113(26):6511-21.
18. Gaman AM, Surlin V, Gaman GD. Splenectomy in Primary Immune Thrombocytopenia (ITP). Prensa Med Argent. 2013;99(10):2.
19. Gaman A, Gaman G. Classic splenectomy versus laparoscopic splenectomy for immune thrombocytopenic purpura in patients with severe refractory thrombocytopenia. Haematologica-the Hematology Journal. 2009;94:529-529.
20. Cooper N, Stasi R, Cunningham-Rundles S, Feuerstein M, Leonard J, Amadori S, et al. The efficacy and safety of B-cell depletion with anti-CD20 monoclonal antibody in adults with chronic immune thrombocytopenic purpura. British Journal of Haematology. 2004;125(2):232-9.
21. Arnold D, Dentali F, Crowther M, Meyer R, Cook R, Sigouin C, et al. Systematic review: Efficacy and safety of rituximab for adults with idiopathic thrombocytopenic purpura. Annals of Internal Medicine. 2007;146(1):25-W5.
22. Zaja F, Vianelli N, Volpetti S, Battista M, Defina M, Palmieri S, et al. Low-dose rituximab in adult patients with primary immune thrombocytopenia. European Journal of Haematology. 2010;85(4):329-34.
23. Bao W, Bussel J, Heck S, He W, Karpoff M, Boulad N, et al. Improved regulatory T-cell activity in patients with chronic immune thrombocytopenia treated with thrombopoietic agents. Blood. 2010;116(22):4639-45.
24. Gaman G, Gaman M. The treatment with eltrombopag of the refractory ITP. Haematologica. 2014;99:207-207.
25. Gaman A, Gaman M. The treatment-associated side effects in patients with idiopathic thrombocytopenic purpura. Haematologica. 2016;101:840-840.
26. Lozano M, Revilla N, Gonzalez-Lopez T, Novelli S, Gonzalez-Porras J, Sanchez- Gonzalez B, et al. Real-life management of primary immune thrombocytopenia (ITP) in adult patients and adherence to practice guidelines. Annals of Hematology. 2016;95(7):1089-98.

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Published

2017-03-19

How to Cite

Găman, M.-A., & Găman, A. M. (2017). Pathophysiology, Diagnosis and Treatment of Immune Thrombocytopenia. International Journal of Medical Students, 5(1), 32–36. https://doi.org/10.5195/ijms.2017.171

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Vol. 5 No. 1 (2017)

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Review

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