Differential diagnosis of thrombocytopenes
https://doi.org/10.17650/1818-8346-2017-12-1-78-87
Abstract
The article presents a systematic diagnostic approach for thrombocytopenia, as well as the results of practical implementation of thrombocytopenia diagnostic protocol in adult patients. Among 183 patients first admitted to HRC with unspecified origin thrombocytopenia, primary immune thrombocytopenia (ITP) was established only in 48 % of patients, while in 52 % of cases it was a symptom of another pathology (the ratio was 1 : 1). As a result of re-examination of 118 relapse patients with long-term history of ITP diagnosis was confirmed in 85 % of cases, in 15 % the diagnosis has been changed to a different nosology.
The results show that to establish the true causes of thrombocytopenia is necessary to conduct an extensive diagnostic search according to presented protocol.
Differential diagnosis between primary immune and secondary thrombocytopenia should be carried out not only in the onset of the disease, but also in the relapse of an earlier established ITP.
About the Authors
A. L. Melikyan
Hematological Research Center;
Russian Federation
4A Noviy Zykovsky Proezd, Moscow 125167, Russia
Russian Federation
4A Noviy Zykovsky Proezd, Moscow 125167, Russia
E. I. Pustovaya
Hematological Research Center;
Russian Federation
4A Noviy Zykovsky Proezd, Moscow 125167, Russia
Russian Federation
4A Noviy Zykovsky Proezd, Moscow 125167, Russia
E. K. Egorova
Hematological Research Center;
Russian Federation
4A Noviy Zykovsky Proezd, Moscow 125167, Russia
Russian Federation
4A Noviy Zykovsky Proezd, Moscow 125167, Russia
M. V. Kalinina
Hematological Research Center;
Russian Federation
4A Noviy Zykovsky Proezd, Moscow 125167, Russia
Russian Federation
4A Noviy Zykovsky Proezd, Moscow 125167, Russia
T. I. Kolosheynova
Hematological Research Center;
Russian Federation
4A Noviy Zykovsky Proezd, Moscow 125167, Russia
Russian Federation
4A Noviy Zykovsky Proezd, Moscow 125167, Russia
I. N. Subortseva
Hematological Research Center;
Russian Federation
4A Noviy Zykovsky Proezd, Moscow 125167, Russia
Russian Federation
4A Noviy Zykovsky Proezd, Moscow 125167, Russia
E. A. Gilyazitdinova
Hematological Research Center;
Russian Federation
4A Noviy Zykovsky Proezd, Moscow 125167, Russia
Russian Federation
4A Noviy Zykovsky Proezd, Moscow 125167, Russia
V. N. Dvirnyk
Hematological Research Center;
Russian Federation
4A Noviy Zykovsky Proezd, Moscow 125167, Russia
Russian Federation
4A Noviy Zykovsky Proezd, Moscow 125167, Russia
References
1. Provan D., Stasi R., Newlnd A.C. et al. International consensus report on the investigation and management of primary immune thrombocytopenia. Blood 2010;115:168–86. DOI: 10.1182/blood-2009-06-225565.
2. Rodeghiero F., Stasi R., Gernsheimer T. et al. Standardization of terminology, definitions and outcome criteria in immune thrombocytopenic purpura of adults and children: report from international working group. Blood 2009;113:2386–93. DOI: 10.1182/blood-2008-07-162503.
3. Cines D.B., Bussel J.B, Liebman H.A., Luning Prak E.T. The ITP syndrome: pathogenic and clinical diversity. Blood 2009;113(26):6511–21. DOI: 10.1182/blood-2009-01-129155.
4. Semple J.W., Milev Y., Cosgrave D. et al. Differences in serum cytokine levels in acute and chronic autoimmune thrombocytopenic purpura: relationship to platelet phenotype and antiplatelet Tcell reactivity. Blood 1996;87:4245–54. PMID: 8639783.
5. Kuhne T. Immune Thrombocytopenia (ITP). 2nd edition. Bremen: UNI-MED, 2012.
6. Kuwana K., Kaburaki J., Ikeda Y. Autoreactive T cells to platelet GPIIb-IIIa in immune thrombocytopenic purpura. Role in production of antiplatelet autoantibody. J Clin Invest 1998;102(7):1393–402. DOI: 10.1172/JCI4238.
7. McMillan R. Autoantibodies and autoantigens in chronic immune thrombocytopenic purpura. Semin Hematol 2000;37:239–48. PMID: 10942218.
8. Mouzaki A., Theodoropoulou M., Gianakopoulos I. et al. Expression patterns of Th1 and Th2 cytokine genes in childhood idiopathic thrombocytopenic purpura (ITP) at presentation and their modulation by intravenous immunoglobulin G (IVIg) treatment: their role in prognosis. Blood 2002;100:1774–9. PMID: 12176899.
9. Johnsen J. Pathogenesis in immune thrombocytopenia: new insights. Hematology Am Soc Hematol Educ Program 2012;2012:306–12. DOI: 10.1182/asheducation-2012.1.306.
10. Sakakura M., Wada H., Tawara I. et al. Reduced Cd4+Cd25+ T cells in patients with idiopathic thrombocytopenic purpura. Thromb Res 2007;120(2):187–93. DOI: 10.1016/j.thromres.2006.09.008.
11. Hu Y., Ma D.X., Shan N.N. et al. Increased number of Tc17 and correlation with Th17 cells in patients with immune thrombocytopenia. PLoS One 2011;6(10):e26522. DOI: 10.1371/journal.pone.0026522.
12. Hu Y., Li H., Zhang L. et al. Elevated profiles of Th22 cells and correlations with Th17 cells in patients with immune thrombocytopenia. Hum Immunol 2012;73(6):629–35. DOI: 10.1016/j.humimm.2012.04.015.
13. Zhu X., Ma D., Zhang J. et al. Elevated interleukin-21 correlated to Th17 and Th1 cells in patients with immune thrombocytopenia. J Clin Immunol 2010;30(2):253–9. DOI: 10.1007/s10875-009-9353-1.
14. Stasi R. How to approach thrombocytopenia. Hematology Am Soc Hematol Educ Program 2012:191–7. DOI: 10.1182/asheducation-2012.1.191.
15. Liebman H.A., Stasi R. Secondary immune thrombocytopenic purpura. Curr Opin Haematol 2007;14(5):557–73. DOI: 10.1097/MOH.0b013e3282ab9904.
16. Aledort L.M., Hayward C.P., Chen M.G. et al. Study Group. Prospective screening of 205 patients with ITP, including diagnosis, serological markers and the relationship between platelet counts, endogenous thrombopoietin, and circulating antithrombopoietin antibodies. Am J Hematol 2004;76(3):205–13. DOI: 10.1002/ajh.20104.
17. Liebman H. Other immune thrombocytopenias. Semin Hematol 2007;44(4 Suppl 5):24–34. DOI: 10.1053/j.seminhematol.2007.11.004.
18. Cines D.B., Liebman H., Stasi R. Pathobiology of secondary immune thrombocytopenia. Semin Hematol 2009;46(1 Suppl 2):2–14. DOI: 10.1053/j.seminhematol.2008.12.005.
19. Меликян А.Л., Пустовая Е.И., Калинина М.В. и др. Диагностический подход при тромбоцитопении. Гематология и трансфузиология 2016;61(1, Приложение1): 23. [Melikyan A.L., Pustovaya E.I., Kalinina M.V. et al. Diagnostic approach for thrombocytopenia. Gematologiya I transfuziologiya = Hematology and Transfusiology 2016; 61(1 Suppl 1)):23 (In Russ.)].
20. Kamath V., Gnanasekaran K.K., Mammen J. MYH9-related disorder, a probable MayHegglin anomaly case series: A tertiary care experience. Hematol Oncol Stem Cell Ther 2016;9(4):137–40. DOI: 10.1016/j.hemonc.2016.08.002.
21. Васильев С.А., Виноградов В.Л., Мазуров А.В., Маркова М.Л. Тромбоцитопении. Акушерство, гинекология и репродукция 2014;(2):112–25. [Vasiliev S.A., Vinogradov V.L., Mazurov A.V., Markova M.L. Thrombocytopenias. Akusherstvo, ginekologiya i reproduktsiya = Obstetrics, gynecology and reproduction 2014;(2):112–25 (In Russ.)].
22. Мазуров А.В. Лабораторная диагностика иммунных тромбоцитопений. В кн.: А.В. Мазуров. Физиология и патология тромбоцитов. М.: Литтерра, 2011. С. 220–222. [Mazurov A.V. Laboratory diagnostic of immune thrombocytopenias. In: A.V. Mazurov. Physiology and patology of platelets. M.: Litterra, 2011. Pp. 220–222 (in Russ.)].
23. Меликян А.Л., Пустовая Е.И., Цветаева Н.В. и др. Национальные клинические рекомендации по диагностике и лечению первичной иммунной тромбоцитопении (идиопатической тромбоцитопенической пурпуры) у взрослых. Гематология и трансфузиология 2015;(1): 44–56. [Melikyan A.L., Pustovaya E.I., Tsvetaeva N.V. et al. National clinical guidelines for the diagnosis and treatment of primary immune thrombocytopenia (idiopathic thrombocytopenic purpura) in adults. Gematologiya I transfuziologiya = Hematology and Transfusiology 2015;(1):44–56 (In Russ.)].
Review
For citations:
Melikyan A.L., Pustovaya E.I., Egorova E.K., Kalinina M.V., Kolosheynova T.I., Subortseva I.N., Gilyazitdinova E.A., Dvirnyk V.N. Differential diagnosis of thrombocytopenes. Oncohematology. 2017;12(1):78-87. (In Russ.) https://doi.org/10.17650/1818-8346-2017-12-1-78-87
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