MUSTAFA ZANYAR AKKUZU, Orhan SEZGİN, OSMAN ÖZDOĞAN, SERKAN YARAŞ, Enver ÜÇBİLEK, Engin ALTINTAŞ, Fehmi ATEŞ, Tuba KARA, MUSTAFA BERKEŞOĞLU

Splenektomi yapılmış hastalarda splenomegalinin nedeni Gaucher hastalığı olabilir mi? 271 splenektomili hastanın incelenmesi

Giriş ve Amaç: Gaucher hastalığı, hepatosplenomegaliye neden olabi-len bir lipit depo hastalığıdır. Zamanında teşhis edilemeyen hastalarda şiddetli splenomegaliye neden olabilir ve teşhis edilmeyen hastalara gereksiz splenektomi yapılabilmektedir. Bu amaçla splenektomi sonrası Gaucher hastalığı olup olmadığını araştırdık. Gereç ve Yöntem: 2010-2019 yılları arasında splenektomi yapılmış toplam 271 hastanın kliniko-patolojik ve demografik verileri geriye dönük olarak incelendi. Bulgular: Splenektominin en sık görülen üç nedeni; travma (%26.5), solid organ tümörleri (%22.88) ve immün trombositopenik purpura (%12.18) idi. Hastaların hiçbirinde Gaucher hastalığından dolayı splenektomi yapıl-mamıştı. Ayrıca tanısal amaçlı splenektomi yapılmış olanların hiçbirinde Gaucher hastalığı saptanmamıştı. Sonuç: Gaucher hastalığında splenek-tomi, hematolojik ve mekanik problemler varken yapılabilmesine rağ-men, artık selektif enzim replasman tedavisi tercih edilen tedavi şeklidir.

Can Gaucher disease be the cause of splenomegaly in patients after splenectomy? Examination of 271 patients with splenectomy

Background and Aims: Gaucher disease is a lipid storage disease that can cause hepatosplenomegaly. Undiagnosed patients may develop se-vere splenomegaly, as well as undergo unnecessary splenectomy. For this purpose, we investigated the presence of Gaucher disease after splenectomy. Materials and Methods: Clinicopathological and demo-graphic data of 271 patients who underwent splenectomy between 2010 and 2019 were retrospectively analyzed. Results: The three most common reasons noted for splenectomy were trauma (26.5%), solid organ tumors (22.88%), and immune thrombocytopenic purpura (12.18%). Conclusion: None of the patients had splenectomy because of Gaucher disease. Moreover, none of the patients who underwent diagnostic splenectomy had Gaucher disease. Although Gaucher dis-ease is associated with splenectomy and hematological and mechanical problems, the preferred treatment modality is selective enzyme replace-ment therapy.

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