Mustafa Zanyar AKKUZU, Orhan SEZGİN, Serkan YARAŞ, Osman ÖZDOĞAN, Enver ÜÇBİLEK, Engin ALTINTAŞ, Fehmi ATEŞ, Tuba KARA, Mustafa BERKEŞOĞLU

271 Splenektomili hastasının incelenmesi, Splenektomi Yapılmış Hastalarda Splenomegalinin Nedeni Gaucher Hastalığı Olabilir mi?

Gaucher hastalığı hepatosplenomegaliye neden olabilen bir lipid depo hastalığıdır. Zamanında tanı konamayan hastalarda ciddi splenomegaliye neden olabilmekte ve tanı alamamış hastalar gereksiz splenektomiye gidebilmektedir. Bu amaçla splenektomi yapılmış hastalar arasında zamanında tanısı konamamış, ancak splenektomi sonrası Gaucher hastalığı tanısı konmuş olanlar var mı diye araştırdık. 2010-2019 yılları arasında splenektomi yapılmış toplam 271 splenektomili hastanın klinikopatolojik ve demografik verileri geriye dönük olarak incelendi. Splenektomi yapılma sebeplerinden en sık üç sebep; travma (%26,5), solid organ tümörlerinin operasyonu esnasında (%22,88) ve immün trombositopenik purpura (%12,18) nedeniyleydi. Hastaların hiçbiri Gaucher hastalığından dolayı splenektomiye verilmemişti ve tanısal amaçlı splenektomi yapılmış olanların hiçbirinde Gaucher hastalığı saptanmamıştı. Gaucher hastalığında splenektomi , hematolojik ve mekanik problemler varken yapılabilmesine rağmen , artık selektif enzim replasman tedavisi tercih edilen tedavi şeklidir.

Anahtar Kelimeler:

Gaucher hastalığı, enzim replasman tedavisi, splenektomi

271 Examination of splenectomy patient, Can the Gaucher Disease be the cause of Splenomegaly in patients with splenectomy ?

Gaucher disease is a lipid storage disease that can cause hepatosplenomegaly. Patients who cannot be diagnosed on time can cause severe splenomegaly and patients who are not diagnosed can undergo unnecessary splenectomy. For this purpose, we investigated the patients who were diagnosed with Gaucher's disease after splenectomy. Clinicopathological and demographic data of 271 patients with splenectomy who underwent splenectomy between 2010-2019 were retrospectively analyzed. The three most common reasons for splenectomy were; trauma (26.5%) was due to the operation of solid organ tumors (22.88%) and immune thrombocytopenic purpura (12.18%). None of the patients were given splenectomy due to Gaucher disease and none of them had Gaucher's disease. None of the 271 patients who underwent splenectomy had no evidence of Gaucher's disease. Although splenectomy, hematological and mechanical problems are present in Gaucher's disease, selective enzyme replacement therapy is the preferred treatment modality.

Keywords:

Gaucher disease enzyme replacement therapy, , splenectomy,

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