Kasabach-Merritt Fenomeni Tablosu ile Kaposiform Hemanjiyoendotelyoma Düşünülen Bir Yenidoğan

Yıl 2017, Cilt: 11 Sayı: 4, 283 - 285, 01.12.2017

Sevim Ünal Deniz Gönülal Hatice Tatar Aksoy Arzu Yazal Erdem Derya Özyörük Ahmet Demir

Öz

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

Öz

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

Anahtar Kelimeler

Kasabach-Merritt phenomenon, Kaposiform hemangioendothelioma, Newborn

Kaynakça

• Cho KH, Kim SH, Park KC, Lee AY, Song KY, Chi JG, et al. Angioblastoma (Nakagawa) - is it the same as tufted angioma? Clin Exp Dermatol 1991;16:110-3.
• Jones EW, Orkin M. Tufted angioma (angioblastoma). A benign progressive angioma, not to be confused with Kaposi’s sarcoma or low-grade angiosarcoma. J Am Acad Dermatol 1989;20:214-25.
• Croteau SE, Liang MG, Kozakewich HP, Alomari AI, Fishman SJ, Mulliken JB, Trenor CC 3rd. Kaposiform hemangioendothelioma: Atypical features and risks of Kasabach-Merritt phenomenon in 107 referrals. J Pediatr 2013;162:142-7.
• Zukerberg LR, Nickoloff BJ, Weiss SW. Kaposiform hemangio- endothelioma of infancy and childhood. An aggressive neoplasm associated with Kasabach-Merritt syndrome and lymphangioma- tosis. Am J Surg Pathol 1993;17:321-8.
• Arai E, Kuramochi A, Tsuchida T, Tsuneyoshi M, Kage M, Fukunaga M, et al. Usefulness of D2-40 immunohistochemistry for differentiation between kaposiform hemangioendothelioma and tufted angioma. J Cutan Pathol 2006;33:492-7.
• Chu CY, Hsiao CH, Chiu HC. Transformation between Kaposiform hemangioendothelioma and tufted angioma. Dermatology 2003; 206:334-7.
• Enjolras O, Wassef M, Mazoyer E, Frieden IJ, Rieu PN, Drouet L, et al. Infants with Kasabach-Merritt syndrome do not have “true” hemangiomas. J Pediatr 1997;130:631-40.
• Ryan C, Price V, John P, Mahant S, Baruchel S, Brandão L, et al. Kasabach-Merritt phenomenon: A single centre experience. Eur J Haematol 2010;84:97-104.
• Kelly M. Kasabach-Merritt phenomenon. Pediatr Clin North Am 2010;57:1085-9.
• Blei F, Karp N, Rofsky N, Rosen R, Greco MA. Successful multimodal therapy for kaposiform hemangioendothelioma complicated by Kasabach-Merritt phenomenon: Case report and review of the literature. Pediatr Hematol Oncol 1998;15:295-305.
• Fuchimoto Y, Morikawa N, Kuroda T, Hirobe S, Kamagata S, Kumagai M, et al. Vincristine, actinomycin D, cyclophosphamide chemotherapy resolves Kasabach-Merritt syndrome resistant to conventional therapies. Pediatr Int 2012;54:285-7.