Yuksel ALTINEL, Ersoy TAŞPINAR, Halil TÜRKAN, Fuat AKSOY, Yılmaz ÖZEN, Rıdvan ALİ

Primer-Kronik İdiyopatik Trombositopenik Purpuralı Tanılı Hastalarda Splenektominin Uzun Dönem Sonuçları: 300 Hastadan Oluşan Tek Merkez Deneyimi

Mevcut çalışma kapsamında, İdiyopatik Trombositopenik Purpuralı (İTP) hastalarında yapılan splenektominin uzun sonuçlarını belirleyerek literatür eşliğinde tartışmak amaçlanmıştır. 2008 ve 2017 yılları arasında Kronik ITP nedeniyle splenektomi yapılan 300 hasta, retrospektif olarak çalışmaya dahil edildi. Veriler, hastaların tıbbi kayıtlarından ve ameliyat notlarından elde edildi. Hastalar, cinsiyet, başvuru anındaki şikayet, majör kanama durumu, trombosit sayıları ve diğer hematolojik parametreler, ilk basamak tedavi kombinasyonları, splenektomi prosedürleri (açık veya laparoskopik), postoperatif trombosit sayıları ve splenektomi ile ilgili olarak, splenektomi sonrası erken ve geç dönem sonuçları, rekürrens, mortalite ve morbidite, splenektomi sonrası üçüncü basamak tedavi ihtiyacı açısından değerlendirildi. Kronik ITP nedeniyle splenektomi yapılan 306 hastanın 6’sı veri eksikliği nedeniyle çalışma dışı bırakıldı. Verisine ulaşılan 300 hasta (88 erkek ve 212 kadın) çalışmaya dahil edildi. 254 (% 84,6) hastada tam yanıt, 2 (% 4) hastada yanıt vardı ve 46 (% 15,3) hastada ise yanıtsızlık vardı. ITP'nin iki yıldaki rekürrens oranı 44 (% 14.6) idi. İTP hem medikal hem de cerrahi tedavilerin uygulanabilirliği açısından en çok görülen hematolojik hastalıklardan biridir ve günümüz tedavi algoritmasında ilk seçenek steroid olarak bilinmektedir. Medikal tedaviye yanıt alınamayan, ilaç yan etkisi gelişen veya yüksek doz steroid ihtiyacı olan hastalarda ise splenektomi halen önemli bir tedavi seçeneği olarak görünmektedir.

Anahtar Kelimeler:

İmmün (idiyopatik) trombositopenik purpura, tedaviye yanıt, splenektomi

Long-Term Results of Splenectomy in Patients Diagnosed with Primary-Chronic Idiopathic Thrombocytopenic Purpura: A Single Center Experience of 300 Patients

The aim of this study was to determine the long results of splenectomy performed in patients with Idiopathic Thrombocytopenic Purpura (ITP) and to discuss them in the light of the literature. Three-hundred patients who underwent splenectomy for chronic ITP between 2008 and 2017 were retrospectively included in the study. Data were obtained from patients' medical records and surgical notes. Patients, gender, complaints at presentation, major bleeding status, platelet counts and other hematological parameters, first-line therapy combinations, splenectomy procedures (open or laparoscopic), postoperative platelet counts and splenectomy, early and late results after splenectomy, recurrence, mortality and morbidity, and the need for tertiary treatment after splenectomy were analyzed. Six of all patients who underwent splenectomy due to chronic ITP were excluded from the study due to lack of data. Thus, 300 patients (88 men and 212 women) were included in the study. 254 (84,6%) patients had complete response, 2 (4%) patients had response, and 46 (15,3%) patients had no response to treatment. The recurrence rate of ITP at two years was 44 (14,6%). ITP is one of the most common hematological diseases in terms of applicability of both medical and surgical treatments and is known as the first choice steroid in today's treatment algorithm. Splenectomy still seems to be an important treatment option in patients who do not respond to medical treatment, develop drug side effects or need high-dose steroids.

Keywords:

Immune (idiopathic) thrombocytopenic purpura, response to treatment, splenectomy,

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1. Stasi R, Evangelista ML, Stipa E, et al. Idiopathic thrombocy-topenic purpura: current concepts in pathophysiology and ma-nagement. Thromb Haemost 2008; 99: 4-13.
2. Stasi R, Provan D. Management of lmmune Thrombocytopenic Purpura in Adults. Mayo Clin Proc 2004; 79: 504 -522.
3. Neunert C, Lim W, Crowther M, Cohen A, Solberg L Jr, Crowther MA (2011) The American Society of Hematology 2011 evidence- based practice guideline for immune throm-bocytopenia. Blood 117:4190–4207
4. Rodeghiero F, Stasi R, Gernsheimer T, Michel M, Provan D, Arnold DM, Bussel JB, Cines DB, Chong BH, Cooper N, God-eau B, Lechner K, Mazzucconi MG, McMillan R, Sanz MA, Imbach P, Blanchette V, Kuhne T, Ruggeri M, George JN (2009) Standardization of terminology, definitions and outcome criteria in immune thrombocytopenic purpura of adults and children: report from an international working group. Blood 113:2386–2393
5. Provan D, Newland A, Norfolk D, Bolton P, Lileyman J, et al. Guidelines for the investigation and management of idiopathic thrombocytopenic purpura in adults, children and in pregnancy. British Journal of Haematology 2003; 20: 574-596.
6. Ismet A, lrfan K, Emin K, M Ali E, Ramazan U, Mustafa B, Onur O. Splenectomy results in patients with idiopathic throm-bocytopenic purpura: 10 years of experience in Turgut Ozal Medical Center. Clin. Lab. Haematology 2004; 26: 211-214.
7. Schwartz J, Leber MD, Gillis SI, Giunta A, Eldor A, Bussel JB. Long¬term follow-up after splenectomy performed for immune thrombocytopenic purpura (ITP). American Journal of Hemato-logy 2003; 72: 94-98.
8. Yenerel MN, Atamer T, Ayer M. Yüz altmış iki kronik idiopa-tik trombositopenik purpura olgusunun klinik seyir ve tedavi cevabı yönünden değerlendirilmesi. İst Tıp Fak Derg 2007; 70: 6-10.
9. Stasi R, Stipa E, Masi M, Cecconi M, Scimo MT, Oliva F, et al. Long-term observation of 208 adults with chronic idiopathic thrombocytopenic purpura. Am J Med 1995; 98: 436-442.
10. Drew Provan, Roberto Stasi, Adrian C. Newland, International consensus report on the investigation and management of pri-mary immune thrombocytopenia bloodjour-nal.hematologylibrary.org by guest on July 23, 2010.
11. Kaya M, Demir C, Esen R, Engin A. Kronik İdiopatik Trombo-sitopenik Purpuralı Olgularımız. Van Tıp Derg 2011: 18(3): 141-146.
12. David PW, Williams DA, Shamberger RC. Immune throm-bocytopenia: surgical therapy and predictors of response. J Pe-diatr Surg 1991; 26(4): 407-13.
13. Stanton CJ. Laparoscopic splenectomy for idiopathic throm-bocytopenic purpura. A five-year experience. Surg Endosc 1999; 13(11): 1083-6.
14. Durakbasa CU, Timur C, Sehiralti V, Mutus M, Tosyali N, Yoruk A. Pediatric splenectomy for hematological diseases: outcome analysis. Pediatr Surg Int 2006; 22(8): 635-9.
15. George JN, Woolf SH, Raskolo GE, et al. ldiopathic throm-bocytopenic purpura: a practice guideline developed by explicit methods for the American Society of Hematology. Blood 1996; 88: 3-40.
16. Stasi R. Pathophysiology and therapeutic options in primary immune thrombocytopenia. Blood Transfus. 2011; 9: 262-73.
17. Türk Hematoloji Derneği Ulusal Tedavi Klavuzu. İmmün trombositopeni tanı ve tedavi klavuzu 2011.
18. Vianelli N, Galli M, De Vivo A, et al. Efficacy and safety of splenectomy in immune thrombocytopenic purpura: Long term results of 402 cases. Hematol J 2005; 90: 72-77.
19. Worrest T. , Cunningham A., Dewey E. Immune Thrombocy-topenic Purpura Splenectomy in the Context of New Medical Therapies , Journal of Surgical Research , January 2020
20. Gupta S. , Kalayarasan R. ,Chandrasekar S. Laparoscopic Splenectomy for Immune Thrombocytopenic Purpura (ITP) Pa-tients with Very Severe Thrombocytopenia , Indian J Hematol Blood Transfus (July-Sept 2018) 34(3):535–539
21. Lambert MP. , Gernsheimer TB. Clinical updates in adult immune thrombocytopenia , Blood 2017.
22. Liebman HA, Pullarkat V. Diagnosis and Management of Immune Thrombocytopenia in the Era of Thrombopoietin Mi-metics, Hematology Am Soc Hematol Educ Program 2011; 2011: 384-390.
23. Neunert C. , Terrell DR. , Arnold DM et al. American Society of Hematology 2019 guidelines for immune thrombocytopenia , blood advances 2019 , DOI 10.1182/ © 2019 by The American Society of Hematology bloodadvances.2019000966