INTRODUCTION Benign vascular tumors are the most com- mon congenital lesions in infancy and childho- od. They are generally superficial subcutaneous tumors and most of them regress gradually du- ring prepubertal period. Such tumors are histo- logically heterogeneous and may show different clinical outcomes. However historically, most of the benign-looking vascular lesions were simply categorized as capillary or cavernous he- mangiomas. Today, they are categorized as dis- tinguishable lesions both architecturally and im- munophenotypically, such as congenital heman- giomas, non-involuting congenital hemangio- ABSTRACT Hemangiomas are the most common congenital lesions in childhood. They are generally superficial tumors and cause a red or violet color change on overlying skin. Most of them gradually regress by time, and become ne- arly imperceptible before puberty, but some bigger and deeper lesions tend to persist in adult life. Although so- me genetic disorders like von Hippel-Lindau syndrome are known to be closely associated with multiple heman- giomas (hemangiomatosis), most of them are sporadic and the etiologic factors have not been clearly elucida- ted. The liver and spleen are two internal organs which can be involved by hemangiomas. Although hemangio- mas are generally harmless lesions, local or systemic unwanted effects can sometimes be seen. Rupture and bleeding caused by rapid enlargement, and hematologi- cal manifestations primarily appearing as thrombocyto- penia, anemia, and coagulopathy may be cited as examples of serious complications. The latter clinico- pathologic condition is manifested by hemorrhagic diat- hesis and is known as Kasabach-Merritt syndrome. The mortality rate is reported to reach one third of the af- fected patients. Key words: Hemangioma, hemangiomatosis, spleen, Kasabach-Merritt Syndrome ÖZET Hemanjiyomlar çocukluk ça¤›n›n en s›k görülen do¤um- sal lezyonlar›d›r. Genellikle yüzeyel yerleflimli tümörler olup, üzerlerindeki deride k›rm›z›-morumsu renk de¤i- flikli¤ine yol açarlar. Ço¤unlu¤unun zaman içinde geri- leyip pubertal geliflimden önce neredeyse silinmesine karfl›n, özellikle büyük ve derin yerleflimli olanlar erifl- kin dönemde de varl›klar›n› sürdürme e¤ilimindedir. von Hippel-Lindau sendromu gibi baz› genetik hastal›k- lar›n multipl hemanjiyomlar (hemanjiyomatozis) ile ilifl- kili oldu¤u bilinse de, ço¤u hemanjiyom sporadiktir ve etiyolojik faktörler kesin olarak ortaya konamam›flt›r. Karaci¤er ve dalak, hemanjiyomlar›n görüldü¤ü iki bü- yük iç organd›r. Hemanjiyomlar genel olarak zarars›z lezyonlar olmalar›na karfl›n, baz› komplikasyonlara ne- den olabilirler. H›zl› büyüme sonucu rüptür ve kanama d›fl›nda trombositopeni, anemi ve koagülopati ile öne ç›- kan baz› hematolojik anormallikler ciddi komplikas- yonlar aras›nda say›labilir. Kanama e¤ilimi ile seyreden bu a¤›r hematolojik tablo Kasabach-Merritt sendromu olarak bilinir ve mortalite oran› etkilenen vakalar›n üç- te birine kadar ulaflabilir. Anahtar sözcükler: Hemanjiyom, hemanjiyomatozis, dalak, Kasabach-Merritt Sendromu A splenic hemangiomatosis case manifested by Kasabach-Merritt syndrome in an adult Eriflkin bir hastada Kasabach-Merritt sendromu ile ortaya ç›kan bir dalak hemanjiyomatozisi olgusu Y›ld›r›m KARSLIO/LU 1 , Ali Fuat Ç‹ÇEK 1 , Mükerrem SAFALI 1 , Ahmet ‹FRAN 2 , Cengiz BEYAN 2 Department of Pathology 1 and Department of Hematology 2 , Gülhane Military Medical Academy and School of Medicine, ANKARA Gelifl tarihi: 27.11.2007 Kabul tarihi: 16.3.2008 This article was presented as a poster in 17th National Congress of Pathology, September 8-13, ‹stanbul, Türkiye Corresponding Author: Dr. Y›ld›r›m Karsl›o¤lu, Gülhane Askeri T›p Akademisi Patoloji Anabilim Dal›, Ankara 107 Turkish Journal of Pathology 2008;24(2):107-110