Önder Özden, Şeref Selçuk Kılıç, Murat Alkan, Banu İnce

Hematolojik hastalığı olan çocuklarda splenektominin endikasyonları ve etkinliği

Amaç: Bu çalışmada çocuklarda hematolojik hastalıklar nedeniyle yapılan splenektominin faydasını ve endikasyonlarının araştırılması amaçlanmıştır.Gereç ve Yöntem: Hastanemizde 2009-2015 yılları arasında, 0-17 yaş aralığında, herhangi bir sebepten dolayı splenektomi yapılmış hastalar araştırıldı. Hastalarının yaşı, cinsiyeti, tanıları, postsplenektomi enfeksiyon olup olmadığı, portal venoz tromboz (PVT) olup olmadığı, ameliyat tipi, preoperatif ve postoperatif beyaz küre sayısı, hemotokrit ve trombosit sayısı, eşzamanlı kolesistektomi, dalak boyutu ve ameliyattan fayda görüp görmediği retrospektif olarak incelendi.Bulgular: Hastaların (n=58) yaş ortancası 8’di. % 41’i kız, % 59 ‘u erkekti. Dört hastada splenektomi sonrası PVT gelişti. Hastaların 8’ine laparoskopik splenektomi yapıldı. 2 hasta ise ameliyata laparoskopik başlanmış ancak kanama sebebiyle açığa dönülmüştür. On dört hastaya eş zamanlı kolesistektomi yapıldı. En çok orak hücreli anemi (n=20 %34,5) ,daha sonra talasemi major ( n=17 %29,3) ve immün trombositopenik purpura (n=6 %10,3) tanılarıyla splenektomi yapıldı. Dokuz (%15) hasta splenektomi sonrası enfeksiyon nedeniyle hastanemize yatışı yapıldı. Hastaların 17 ‘sinde splenektomiden fayda görüp görmediği veri yetersizliğinden dolayı değerlendirilemedi. Değerlendirilen hastaların %85’i (n=35) splenektomiden fayda gördü. 4 hasta eksitus olmuştur. Talasemi major ve İTP olan birer hasta splenektomiden fayda görmedi. Orak hücreli anemi olan hastaların hepsi splenektomiden fayda gördü.Sonuç: Splenektomi, hala birçok hematolojik hastalığın tedavisi için kullanılan etkili cerrahi bir işlemdir. Trombosit sayısını yükseltmek için yapılan ameliyatlarda hastanın yaşı, azda olsa önemlidir.

Anahtar Kelimeler:

splenektomi, çocuk, hematolojik hastalıklar

Indication and efficacy of splenectomy in children with hematological disease

Purpose:. We aimed to research benefits and indications of splenectomy operation due to hematological disease in children.Materials and Methods: The children who underwent to splenectomy due to hematological diseases between 2009-2015 were included in this study. Age of patients, sex, diagnosis, post splenectomy infection, portal vein thrombosis (PVT), operation types, pre and post operative thrombocyte, leucocyte and hematocrit counts, simultaneous cholecystectomy, spleen size, the efficacy of operation were noted retrospectively. Results: Median age of the patients (n=58) was 8. 41% of them were girl and 51% were boy. PVT occurred in 4 patients after splenectomy. 8 of them underwent splenectomy laparoscopically. 2 had conversion due to uncontrolled bleeding. 14 patients underwent cholecystectomy simultaneously. Diagnosis were sickle cell anemia (SCA) (n=20, 34.5%), thalassemia major (n=17, 29.3%) and immune thrombocytopenic purpura (ITP) (n=6, 10.3%). 9 (15%) patients were hospitalized because of post splenectomy infections. Efficacy of splenectomy was not evaluated in 17 patients due to data lack. Efficacy of splenectomy was 85% in evaluated patients. 4 patients were death. One each of thalassemia major and ITP patients had no benefit and all of SCA patients had benefited from splenectomy. Conclusion: Splenectomy is still efficient operation procedure for many hematologic diseases. Age of patients is also important in respect of increasing thrombocyte counts

Keywords:

splenectomy, children, hematological diseases,

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1. Rescorla FJ, West KW, Engum SA, Grosfeld JL. Laparoscopic splenic procedures in children: experience in 231 children. Ann Surg. 2007;246:683-7.
2. Murawski M, Patkowski D, Korlacki W, Czauderna P, Sroka M, Makarewicz W et al. Laparoscopic splenectomy in children--a multicenter experience. J Pediatr Surg. 2008;43:951-4.
3. Wilkins BS. The spleen. Br J Haematol. 2002;117:265-74.
4. Rodeghiero F, Ruggeri M. Short- and long-term risks of splenectomy for benign haematological disorders: should we revisit the indications? Br J Haematol. 2012;158:16-29.
5. Emond AM, Collis R, Darvill D, Higgs DR, Maude GH, Serjeant GR. Acute splenic sequestration in homozygous sickle cell disease: natural history and management. J Pediatr. 1985;107:201-6.
6. Rice HE, Englum BR, Rothman J, Leonard S, Reiter A, Thornburg C et al. Clinical outcomes of splenectomy in children: report of the splenectomy in congenital hemolytic anemia registry. Am J Hematol. 2015;90:187-92.
7. Chong BH. Diagnosis, treatment and pathophysiology of autoimmune thrombocytopenias. Crit Rev Oncol Hematol. 1995;20:271-96.
8. Cohen A, Gayer R, Mizanin J. Long-term effect of splenectomy on transfusion requirements in thalassemia major. Am J Hematol. 1989;30:254-6.
9. Borzi P. Splenectomy. In Pediatric Surgery. (Ed MEH Prem Puri):403-10. Germany, Springers, 2006.
10. Iolascon A, Andolfo I, Barcellini W, Corcione F, Garcon L, De Franceschi L et al. Recommendations regarding splenectomy in hereditary hemolytic anemias. Haematologica. 2017;102:1304-13.
11. Horowitz J, Smith JL, Weber TK, Rodriguez-Bigas MA, Petrelli NJ. Postoperative complications after splenectomy for hematologic malignancies. Ann Surg. 1996;223:290-6.
12. Davies IL, Cho J, Lewis MH. Splenectomy results from an 18-year single centre experience. Ann R Coll Surg Engl. 2014;96:147-50.
13. Bickenbach KA, Gonen M, Labow DM, Strong V, Heaney ML, Zelenetz AD et al. Indications for and efficacy of splenectomy for haematological disorders. Br J Surg. 2013;100:794-800.
14. Bai YN, Jiang H, Prasoon P. A meta-analysis of perioperative outcomes of laparoscopic splenectomy for hematological disorders. World J Surg. 2012;36:2349-58.
15. Bolton-Maggs PH, Stevens RF, Dodd NJ, Lamont G, Tittensor P, King MJ et al. Guidelines for the diagnosis and management of hereditary spherocytosis. Br J Haematol. 2004;126:455-74.
16. Eraklis AJ, Kevy SV, Diamond LK, Gross RE. Hazard of overwhelming infection after splenectomy in childhood. N Engl J Med. 1967;276:1225-9.
17. Styrt B. Infection associated with asplenia: risks, mechanisms, and prevention. Am J Med. 1990;88:33N-42N.
18. Kristinsson SY, Gridley G, Hoover RN, Check D, Landgren O. Long-term risks after splenectomy among 8,149 cancer-free American veterans: a cohort study with up to 27 years follow-up. Haematologica. 2014;99:392-8.
19. Ahmed R, Devasia AJ, Viswabandya A, Lakshmi KM, Abraham A, Karl S et al. Long-term outcome following splenectomy for chronic and persistent immune thrombocytopenia (ITP) in adults and children : Splenectomy in ITP. Ann Hematol. 2016;95:1429-34.
20. Krauth MT, Lechner K, Neugebauer EA, Pabinger I. The postoperative splenic/portal vein thrombosis after splenectomy and its prevention--an unresolved issue. Haematologica. 2008;93:1227-32.
21. Robinette CD, Fraumeni JF, Jr. Splenectomy and subsequent mortality in veterans of the 1939-45 war. Lancet. 1977;2:127-9.
22. Winslow ER, Brunt LM, Drebin JA, Soper NJ, Klingensmith ME. Portal vein thrombosis after splenectomy. Am J Surg. 2002;184:631-5; discussion 5-6.
23. Brink JS, Brown AK, Palmer BA, Moir C, Rodeberg DR. Portal vein thrombosis after laparoscopy-assisted splenectomy and cholecystectomy. J Pediatr Surg. 2003;38:644-7.
24. Soyer T, Ciftci AO, Tanyel FC, Senocak ME, Buyukpamukcu N. Portal vein thrombosis after splenectomy in pediatric hematologic disease: risk factors, clinical features, and outcome. J Pediatr Surg. 2006;41:1899-902.
25. Ikeda M, Sekimoto M, Takiguchi S, Yasui M, Danno K, Fujie Y et al. Total splenic vein thrombosis after laparoscopic splenectomy: a possible candidate for treatment. Am J Surg. 2007;193:21-5.
26. Arnoletti JP, Karam J, Brodsky J. Early postoperative complications of splenectomy for hematologic disease. Am J Clin Oncol. 1999;22:114-8.
27. Musser G, Lazar G, Hocking W, Busuttil RW. Splenectomy for hematologic disease. The UCLA experience with 306 patients. Ann Surg. 1984;200:40-5