Refractory Immune Thrombocytopenic Purpura and Cytomegalovirus Infection: A Call for a Change in the Current Guidelines

Main Article Content

Alex Shimanovsky
Devbala Patel
Jeffrey Wasser

Keywords

Immune Thrombocytopenic Purpura, ITP, Cytomegalovirus, CMV, Refractory ITP, CMV-associated ITP

Abstract

Immune thrombocytopenic purpura (ITP) is characterized by a decreased platelet count caused by excess destruction of platelets and inadequate platelet production. In many cases the etiology is not known, but viral illness is thought to play a role in the development of some cases of ITP. The current (2011) American Society of Hematology ITP guidelines recommend initial diagnostic studies to include testing for HIV and Hepatitis C. The guidelines suggest that initial treatment consist of observation, therapy with corticosteroids, IVIG or anti D. While most cases respond to the standard therapy such that the steroids may be tapered and the platelet counts remain at a hemostatically safe level. Some patients with ITP are dependent on long term steroid maintenance and the thrombocytopenia persists with the tapering of the steroids. Recent case reports demonstrate that ITP related to cytomegalovirus (CMV) can persist in spite of standard therapy and that antiviral therapy maybe indicated. Herein we report a case of a 26-year-old female with persistent ITP that resolved after the delivery of a CMV infected infant and placenta. Furthermore we review the current literature on CMV-associated ITP and propose that the current ITP guidelines be amended to include assessment for CMV as part of the work-up for severe and refractory ITP prior to splenectomy.

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References

1. Wright JF, Blanchette VS, Wang H, et al.: Characterization of platelet-reactive antibodies in children with varicella-associated acute immune thrombocytopenic purpura (ITP). Br J Haematol 1996, 95:145-152.

2. Alliot C, Barrios M: Cytomegalovirus-induced thrombocytopenia in an immunocompetent adult effectively treated with intravenous immunoglobulin: a case report and review. Hematology 2005, 10:277-279.

3. Sahud MA, Bachelor MM: Cytomegalovirus-induced thrombocytopenia. An unusual case report. Arch Intern Med 1978, 138:1573-1575.

4. Sugioka T, Kubota Y, Wakayama K, et al.: Severe steroid-resistant thrombocytopenia secondary to cytomegalovirus infection in an immunocompetent adult. Intern Med 2012, 51:1747-1750.

5. van Spronsen DJ, Breed WP: Cytomegalovirus-induced thrombocytopenia and haemolysis in an immunocompetent adult. Br J Haematol 1996, 92:218-220.

6. DiMaggio D, Anderson A, Bussel JB: Cytomegalovirus can make immune thrombocytopenic purpura refractory. Br J Haematol 2009, 146:104-112.

7. Griffith BP, McCormick SR, Fong CK, et al.: The placenta as a site of cytomegalovirus infection in guinea pigs. J Virol 1985, 55:402-409.

8. Wright JG: Severe thrombocytopenia secondary to asymptomatic cytomegalovirus infection in an immunocompetent host. J Clin Pathol 1992, 45:1037-1038.

9. Sheng Yu Z, Tang LF, Zou CC, et al.: Cytomegalovirus-associated idiopathic thrombocytopenic purpura in Chinese children. Scand J Infect Dis 2008, 40:922-927.

10. Ding Y, Zhao L, Mei H, et al.: Role of myeloid human cytomegalovirus infection in children's idiopathic thrombocytopenic purpura. Pediatr Hematol Oncol 2007, 24:179-188.

11. Shimm DS, Logue GL, Rosse WF: Recurrent thrombocytopenia following idiopathic thrombocytopenic purpura. The importance of platelet-bound IgG in establishing cause. Arch Intern Med 1980, 140:855-857.

12. Gural A, Gillis S, Gafanovich A, et al.: Massive intracranial bleeding requiring emergency splenectomy in a patient with CMV-associated thrombocytopenia. Haemostasis 1998, 28:250-255.

13. Arruda VR, Rossi CL, Nogueira E, et al.: Cytomegalovirus infection as cause of severe thrombocytopenia in a nonimmunosuppressed patient. Acta Haematol 1997, 98:228-230.

14. Shreshta Rajsha RD, Sherpa Mingma: Cytomegalovirus: A possible cause of Persistent Refractory Immune Thrombocytopenic Purpura. Journal of Advances in Internal Medicine 2014, 3:42-45.

15. Papagianni A, Economou M, Tsoutsou E, et al.: CMV-related immune thrombocytopenic purpura or CMV-induced thrombocytopenia? Br J Haematol 2010, 149:454-455.

16. Verdonck LF, de Gast GC, van Heugten HG, et al.: Cytomegalovirus infection causes delayed platelet recovery after bone marrow transplantation. Blood 1991, 78:844-848.

17. Psaila B, Bussel JB: Refractory immune thrombocytopenic purpura: current strategies for investigation and management. Br J Haematol 2008, 143:16-26.

18. Neunert C, Lim W, Crowther M, et al.: The American Society of Hematology 2011 evidence-based practice guideline for immune thrombocytopenia. Blood 2011, 117:4190-4207.