İmmün Trombositopenik Purpura Hastalarının Retrospektif Analizi

Abstract

Amaç: Kronik immün trombositopenik purpura (ITP) tanısı konan ve/veya izlenen hastaların klinik özellikleri ve tedavi yanıtları değerlendirdik. Yöntemler: Ondokuz Mayıs Tıp Fakültesi'nde 2003-2011 yılları arasında ITP tanısı konan ve/veya takibi yapılan 150 hastanın tıbbi kayıtları geriyedönük incelendi. Birinci basamak tedavi olarak steroid bazlı tedavi ve IVIg kullanıldı. Birinci ve ikinci basamak tedavilerine cevap vermeyen hastalara splenektomi uygulandı. Bulgular: 150 hastanın ortanca takip süresi 15 ay (aralık 2-83 ay) idi. Olguların 51'inde (%34) trombositopeni tesadüfen saptanmıştı. Çalışma süresi boyunca 21 (%14) hasta tedavisiz takip edildi. 129 hastaya (%86) birinci basamak tıbbi tedavi verildi. Bunlardan 93'ünde (%72) tam yanıt (14), 14'ünde (%11) kısmi yanıt (PR) ve 22 (%17) hastada herhangi bir yanıt görülmedi (NR). Yüksek doz steroid tedavisinin standart doz tedavisine göre anlamlı bir faydası yoktu (p = 0,59). Tedaviye yanıt veren 107 hastanın 2,5'inde 48 (%45) nüks görüldü. 40 hastanın 15'inde (%38) CR, 9'unda (%22) PR ve 16'sında (%40) NR vardı. 38 hastaya splenektomi uygulandı. 48 hastadan 32'sinde (%84) CR, 2'sinde (%5) PR ve 4'ünde (%16) NR elde edildi. On iki hastada (%35) nüks görüldü. Sonuç: Bu çalışma ITP hastalarında steroid bazlı tedavi ve splenektominin çok etkili bir tedavi olduğunu göstermiştir. Çalışmamızda sadece 13 (%8,6) hasta daha ileri tedaviye ihtiyaç duydu.

Keywords

• İmmün Trombositopenik Purpura,kanama,kortikosteroid,splenektomi

Retrospective Analysis of Patients with Immune Thrombocytopenic Purpura

Abstract

Aim: We evaluated the clinical feature and the responses to treatments in patients diagnosed and/or followed as chronic immune thrombocytopenic purpura (ITP). Methods: Medical charts of 150 patients diagnosed and/or followed as ITP at Ondokuz Mayıs Medical Faculty between 2003 and 2011 were analyzed retrospectively. As first-line treatments, steroids based treatment and IVIg were used as medical therapy. In patients, who had no response to first and second courses treatments, splenectomy was performed. Results: The median follow-up of 150 patients was 15 months (range 2-83 months). Thrombocytopenia was incidentally detected in 51(34%) of the cases. During the study period, 21(14%) the patients were followed up without treatment. First line medical therapy were given to 129(86%) patients. Of them, complete response (CR) were seen in 93(72%) patients, partial response (PR) in 14(11%) patients and none response (NR) in 22 (17%) patients. There was no significant benefit of high dose steroid therapy over the standard dose therapy (p=0.59). Of the 107 patients who had response to the treatment, relapse were observed in 48(45%) within 2,5 years. Of 40 patients, there were CR in 15(38%) patients, PR in 9(22%) patients and NR in 16(40%) patients. Splenectomy was performed in 38 patients. Of the 48 patients, CR was achieved in 32(84%) patients, PR in 2(%5) patients and NR in 4(16%) patients. Relapse was observed in 12(35%) patients. Conclusion: This study showed that steroids based treatment and splenectomy are very effective treatment in ITP patients. Only 13(8.6%) patients in our study needed further treatment.

Keywords

• Immune Thrombocytopenic Purpura,Bleeding,corticosteroid,splenectomy

References

1. Reference 1. Segal JB, Powe NR. Prevalence of immune thrombocytopenia: analyses of administrative data. Journal of thrombosis and haemostasis. 2006;4(11):2377-83.
2. Reference 2. Guthrie TH, Jr., Brannan DP, Prisant LM. Idiopathic thrombocytopenic purpura in the older adult patient. The American journal of the medical sciences. 1988;296(1):17-21.
3. Reference 3. Cines DB, Bussel JB. How I treat idiopathic thrombocytopenic purpura (ITP). Blood. 2005;106(7):2244-51.
4. Reference 4. Matzdorff A, Arnold G. Treatment of chronic immune thrombocytopenic purpura: the patients' perspective. European journal of haematology. 2007;78(5):381-8.
5. Reference 5. Portielje JE, Westendorp RG, Kluin-Nelemans HC, Brand A. Morbidity and mortality in adults with idiopathic thrombocytopenic purpura. Blood. 2001;97(9):2549-54.
6. Reference 6. Vianelli N, Valdre L, Fiacchini M, de Vivo A, Gugliotta L, Catani L, et al. Long-term follow-up of idiopathic thrombocytopenic purpura in 310 patients. Haematologica. 2001;86(5):504-9.
7. Reference 7. Pamuk GE, Pamuk ON, Baslar Z, Ongoren S, Soysal T, Ferhanoglu B, et al. Overview of 321 patients with idiopathic thrombocytopenic purpura. Retrospective analysis of the clinical features and response to therapy. Annals of hematology. 2002;81(8):436-40.
8. Reference 8. Cortelazzo S, Finazzi G, Buelli M, Molteni A, Viero P, Barbui T. High risk of severe bleeding in aged patients with chronic idiopathic thrombocytopenic purpura. Blood. 1991;77(1):31-3.

9. Reference 9. Mithoowani S, Arnold DM. First-Line Therapy for Immune Thrombocytopenia. Hamostaseologie. 2019;39(3):259-65.
10. Reference 10. Marini I, Bakchoul T. Pathophysiology of Autoimmune Thrombocytopenia Current Insight with a Focus on Thrombopoiesis. Hamostaseologie. 2019;39(3):227-37.
11. Reference 11. Samson M, Fraser W, Lebowitz D. Treatments for Primary Immune Thrombocytopenia: A Review. Cureus 2019;11(10):e5849.
12. Reference 12. Matschke J, Müller-Beissenhirtz H, Novotny J, et al. A Randomized Trial of Daily Prednisone versus Pulsed Dexamethasone in Treatment-Naïve Adult Patients with Immune Thrombocytopenia: EIS 2002 Study. Acta Haematol 2016;136(2):101-7.
13. Reference 13. 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.
14. Reference 14. Dameshek W, Rubio F, Jr., Mahoney JP, Reeves WH, Burgin LA. Treatment of idiopathic thrombocytopenic purpura (ITP) with prednisone. Journal of the American Medical Association. 1958;166(15):1805-15.
15. Reference 15. Mazzucconi MG, Francesconi M, Fidani P, Di Nucci G, Gandolfo GM, Afeltra A, et al. Treatment of idiopathic thrombocytopenic purpura (ITP): results of a multicentric protocol. Haematologica. 1985;70(4):329-36.
16. Reference 16. Altintop L, Albayrak D. Oral high-dose methylprednisolone and intravenous immunoglobulin treatments in adult chronic idiopathic thrombocytopenic purpura. American journal of hematology. 1997;56(3):191-2.
17. Reference 17. Godeau B, Zini JM, Schaeffer A, Bierling P. High-dose methylprednisolone is an alternative treatment for adults with autoimmune thrombocytopenic purpura refractory to intravenous immunoglobulins and oral corticosteroids. American journal of hematology. 1995;48(4):282-4.
18. Reference 18. Alpdogan O, Budak-Alpdogan T, Ratip S, Firatli-Tuglular T, Tanriverdi S, Karti S, et al. Efficacy of high-dose methylprednisolone as a first-line therapy in adult patients with idiopathic thrombocytopenic purpura. British journal of haematology. 1998;103(4):1061-3.
19. Reference 19. den Ottolander GJ, Gratama JW, de Koning J, Brand A. Long-term follow-up study of 168 patients with immune thrombocytopenia. Implications for therapy. Scandinavian journal of haematology. 1984;32(1):101-10.
20. Reference 20. Shashaty GG, Rath CE. Idiopathic thrombocytopenic purpura in the elderly. The American journal of the medical sciences. 1978;276(3):263-7.
21. Reference 21. George JN1, Woolf SH, Raskob GE, et al. Idiopathic thrombocytopenic purpura: a practice guideline developed by explicit methods for the American Society of Hematology. Blood 1996;88(1):3-40.
22. Reference 22. Depre F, Aboud N, Mayer B, Salama A. Efficacy and tolerability of old and new drugs used in the treatment of immune thrombocytopenia: results from a long-term observation in clinical practice. PLoS One. 2018;13(6):e0198184.
23. Reference 23. Guan Y, Wang S, Xue F, et al. Long-term results of splenectomy in adult chronic immune thrombocytopenia. Eur J Haematol 2017;98(3):235-41.
24. Reference 24. Guidelines for the investigation and management of idiopathic thrombocytopenic purpura in adults, children and in pregnancy. British journal of haematology. 2003;120(4):574-96.
25. Reference 25. Quiquandon I, Fenaux P, Caulier MT, Pagniez D, Huart JJ, Bauters F. Re-evaluation of the role of azathioprine in the treatment of adult chronic idiopathic thrombocytopenic purpura: a report on 53 cases. British journal of haematology. 1990;74(2):223-8.