Akut immün trombositopenik purpura tanısı alan hastaların retrospektif değerlendirilmesi ve yüksek doz metilprednizolon ile intravenöz immunglobulin tedavilerinin karşılaştırılması

Year 2019, , 303 - 308, 01.09.2019

Mahmut Aslan , Ünsal Özgen Neslian Aslan

https://doi.org/10.21601/ortadogutipdergisi.482032

Abstract

Amaç: Çocukluk çağında en sık görülen edinsel trombositopeni nedenlerinden birisi immün trombositopenik purpuradır. İmmün trombositopenik purpura (İTP), otoantikorla kaplı trombositlerin retiküloendotelyal sistemde doku makrofajları aracılığıyla yıkılarak dolaşımdaki trombosit sayısının azalması ve bu otoantikorların trombosit üretimini inhibe etmesi ile seyreden otoimmün bir hastalıktır. Bu çalışmada akut immün trombositopenik purpura tanısı almış hastaların dosyaları retrospektif olarak incelendi. Hastaların başvuru sırasındaki klinik özellikleri ve tedavi seçiminin etkinlikleri belirlenmeye çalışıldı.
Gereç ve Yöntemler: Hastanemiz Çocuk Hematoloji Kliniğinde, Ocak 2010 - Ağustos 2014 tarihleri arasında akut immün trombositopenik purpura tanısı alan hastalar çalışmaya dâhil edildi. Hasta dosyaları retrospektif olarak incelendi. Yüksek doz metilprednizolon ve intravenöz immunglobulin (İVİG) tedavileri alan hastaların tedaviye yanıtları karşılaştırıldı.
Bulgular: Çalışmamıza alınan hastaların 91’i (%56,9) erkek idi. Hastaların %48,13’ünde başvurudan 1-4 hafta öncesinde geçirilmiş enfeksiyon öyküsü mevcuttu. Hastaların ortalama trombosit sayısı 12081±11912 /mm3 idi. Yüz beş (%65,6) hastaya yüksek doz metilprednizolon, 55 (%34,4) hastaya İVİG tedavileri verilmişti ve tedavinin 10. gününde, tedaviye yanıt açısından istatistiksel olarak yüksek doz metilprednizolon ile intravenöz immunglobulin arasında anlamlı fark saptanmadı (p=0,732). Altı ayın sonunda yüksek doz metilprednizolon verilen hastalar ile intravenöz immunglobulin verilen hastalar arasında kronikleşme açısından anlamlı ilişki saptanmadı (p=0,468). Başvuru anında bakılan kanama zamanı 7 dakikanın üzerinde olan %15 hastanın kronikleşme gelişme oranı daha yüksek saptandı (p=0,031).
Sonuç: İmmün trombositopenik purpura çocukluk çağında sık görülen bir hastalık olup hastaların %20-30’u kronikleşmektedir. Yüksek doz metilprednizolon ile intravenöz immunglobulin tedavilerinin kronikleşme üzerine etkisi açısından aralarında fark saptanmamıştır.

Keywords

Çocukluk, İmmün Trombositopenik Purpura, İntravenöz İmmunglobulin, Metilprednizolon

The retrospective evaluation of patients diagnosed with acute immune thrombocytopenic purpura and comparison of treatments of high-dose methylprednisolone and intravenous immunoglobulin

Abstract

Objective: One of the most common causes for acquired thrombocytopenia in childhood is immune thrombocytopenic purpura (ITP) which is an autoimmune disease characterized by decreased platelet counts and inhibition of platelet production due to antibody-mediated destruction of platelets by macrophages in the reticulo endothelial system. In this study, we aimed to compare high-dose methylprednisolone (HDMP) and intravenous immunoglobulin (IVIG) in the treatment ITP.
Materials and Methods: The medical records of 160 patients who were admitted to our Pediatric Hematology Department with the diagnosis of acute ITP between January 2010 and August 2014 were retrospectively analyzed. Treatment responses to HDMP and IVIG were compared.
Results: Of the patients, 91 (56.9%) were males. A total of 48.13% of them had a previous history of infection for one to four weeks before admission. The mean platelet count was 12.081±11.912 /mm3. A total of 105 patients received HDMP, while 55 patients received IVIG. There was no statistically significant difference in the treatment response to HDMP or IVIG on the 10th day of the treatment (p=0.732). At the end of six months, there was no significant difference between the patients receiving HDMP and IVDG in terms of progression to chronicity (p=0.468). The rate of chronicity and relapses was found to be statistically significantly higher in 15% of the patients with a bleeding time of more than 7 minute at the time of arrival (p=0.031).
Conclusion: Based on our study results, we suggest that HDMP and IVIG treatments have similar effects on disease progression to chronicity.

Keywords

Childhood, Immune Thrombocytopenic Purpura, Intravenous Immunoglobulin, Methylprednisolon

References

• Jung JY, O AR, Kim JK, Park M. Clinical course and prognostic factors of childhood immune thrombocytopenia: single center experience of 10 years. Korean J Pediatr 2016; 59(8): 335-340.
• Provan D, Newland AC. Primary immune thrombocytopenia. In: Hoffbrand AV, Catovsky D, Tuddenham E, Green AR, editors. Postgraduate Haematology. 6th ed. London: Wiley-Blackell; 2011. p. 928-939.
• Imbach P. Idiopathic Thrombocytopenic Purpura. In: Arceci RJ, Hann IM, Smith OP, editors. Pediatric Hematology. 3th ed. Oxford: Blackwell Publishing; 2006. p. 526-547.
• Cines DB, Blanchette VS. Immune thrombocytopenic purpura. N Engl J Med 2002; 346(13): 995-1008.
• Glanz J, France E, Xu S, Hayes T, Hambidge S. A population-based, multisite cohort study of the predictors of chronic idiopathic thrombocytopenic purpura in children Pediatrics 2008; 121(3): e506-512.
• Segel GB, Feig SA. Controversies in the diagnosis and management of childhood acute immune thrombocytopenic purpura. Pediatr Blood Cancer 2009; 53(3): 318-324.
• Bolton-Maggs PH, Moon I. Assesment of UK practice for management of acute childhood idiopathic thrombocytopenic purpura against published guidelines. Lancet 1997; 350(9078): 620-623.
• Osborn LM, Neunert CE. Management of newly diagnosed immune thrombocytopenia: can we change outcomes? Blood Adv 2017; 1(24): 2295-2301.
• Kühne T, Buchanan GR, Zimmerman S, Michaels LA, Kohan R, Berchtold W, ve ark. A prospective comparative study of 2540 infants and children with newly diagnosed idiopathic thrombocytopenic purpura (ITP) from the Intercontinental Childhood ITP Study Group. J Pediatr 2003; 143(5): 605-608.
• Blanchette V, Imbach P, Andrew M, Adams M, McMillan J, Wang E, ve ark. Randomised trial of intravenous immunoglobulin G, intravenous anti-D, and oral prednisone in childhood acute immune thrombocytopenic purpura. Lancet 1994(8924); 344: 703-707.
• Medeiros D, Buchanan GR. Major hemorrhage in children with idiopathic thrombocytopenic purpura: immediate response to therapy and long-term outcome. J Pediatr 1998; 133(3): 334-339.
• Wilson DB. Acquired Platelet Defects. Immun Thrombocytopenic Purpura. In: Nathan DG, Orkin SH, Gingsburg D, Look TA, editors. Nathan and Oski’s Hematology of İnfancy and Childhood. 7th ed. Philadelphia: WB Saunders Company; 2009. p. 1557-1565.
• Imbach P. Idiopathic Thrombocytopenic Purpura. In: Arceci RJ, Hann IM, Smith OP, editors. Pediatric Hematology. 3th ed. Oxford: Blackwell Publishing; 2006. p. 526-547.
• Ozsoylu S. High dose intravenous methylprednisolone for chronic idiopathic thrombocytopenic purpura. Acta Haematol 1989; 81(2): 112-113.
• Blanchette VS, Carcao M. Childhood acute immune thrombocytopenic purpura: 20 Years Later. Semin Thromb Hemost 2003; 29(6): 605-617.
• Güngör T, Arman Bilir Ö, Koşan Çulha V, Güngör A, Kara A, Azık FM, Yaralı HN. Retrospective evaluation of children with immune thrombocytopenic purpura and factors contributing to chronicity. Pediatr Neonatol. 2018 Nov 3.