Derya ÖZYÖRÜK, Ahmet DEMİR, Hatice TATAR AKSOY, Sevim ÜNAL, Deniz GÖNÜLAL, Arzu YAZAL ERDEM

Kasabach-Merritt Fenomeni Tablosu ile Kaposiform Hemanjiyoendotelyoma Düşünülen Bir Yenidoğan

Kasabach-Merritt fenomeni (KMF) tüketim koagulopatisi, trombositopeni ve hipofibrinojenemi ile karakterize, nadir görülen bir klinik tablodur. Sıklıkla kaposiform hemanjiyoendotelyoma (KHE) ve küme şeklindeki anjiyomalar olmak üzere vasküler tümörlerle birlikte görülür.Tüm vücutta yaygın peteşi, trombositopeni, lumbal küçük bir kitlesi olan term kız bebek, 5 günlükken hastanemize KMF ön tanısıyla sevk edildi. Fizik muayenesinde sağ inguinalde hematom, yaygın peteşi ve sırtta geniş ekimozu vardı. Klinik ve laboratuvar olarak DIK düşünülen bebeğin magnetik rezonans görüntülemesinde retroperitoneal bölgede 86x54x76 mm, aorta ve sağ böbreği anteriora iten, vertebrayı invaze ederek spinal kanala uzanan dev kavernöz hemanjiyomla uyumlu kitle saptandı. Olgumuzda kitle, KMF ve DIK tabloları nedeniyle KHE düşünüldü. DIK nedeniyle biyopsi yapılamadı ve destek tedavi uygulandı. Pulse-steroid, vinkristin ve aktinomisin-D tedavileri verilen olguda kitlenin küçülmesi sağlandı. Hasta takip ve tedavisine ayaktan devam edilmek üzere 2 aylıkken taburcu edildi. Vasküler tümörlerde KMF ve DIK gelişirse, kanamaya neden olarak mortalite ve morbidite riskini artırabilir. Bu malign klinik tablonun görüldüğü olgularda KHE düşünülmeli, tedavi buna göre planlanarak DIK ve trombositopeni kontrol altına alınmaya çalışılmalıdır.

Anahtar Kelimeler:

Kasabach-Merritt fenomeni, Kaposiform hemanjiyoendotelyoma, Yenidoğan

Kasabach-Merritt Fenomeni Tablosu ile Kaposiform Hemanjiyoendotelyoma Düşünülen Bir Yenidoğan

The Kasabach-Merritt phenomenon (KMP) is a rare clinical condition characterized by consumptive coagulopathy, thrombocytopenia, and hypofibrinogenemia. KMP frequently accompanies vascular tumors such as kaposiform hemangioendothelioma (KHE) and tufted angioma.A term female infant with diffuse petechiae, thrombocytopenia and a small lumbar mass was referred to our hospital on the 5th day with the diagnosis of KMP. Physical examination revealed generalized petechiae, hematoma on the right inguinal region, and a large ecchymosis in the lumbar region. She was diagnosed with disseminated intravascular coagulation (DIC) and supportive treatment was provided. Magnetic resonance imaging revealed a 86x54x76 mm retroperitoneal mass consistent with cavernous hemangioma pushing the aorta and right kidney anteriorly, invading the vertebra and extending to the spinal canal. Biopsy could not be obtained due to severe DIC. KHE was considered with the clinical findings of a giant vascular mass, KMP, and severe DIC. The patient was managed with pulse steroid, vincristine and actinomycin-D treatments. She was discharged at 2 months as the mass became smaller and KMP improved.If KMP and DIC develop in vascular tumors, the risk of bleeding may increase mortality and morbidity. In this malignant clinical condition, KHE should be considered and therapy should be planned accordingly. DIC and thrombocytopenia should be managed by supportive therapies

Keywords:

Kasabach-Merritt phenomenon, Kaposiform hemangioendothelioma, Newborn,

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