Neonatal Kasabach-Merritt Syndrome (KMS): case report
Abstract
A 2days baby girl presented with congenital reddish-blue swelling over chest and abdomen with thrombocytopenia. The clinical, imagining and laboratory findings suggested the diagnosis of KMS. Oral steroid was started with initially needed platelet and FFP transfusions. Large thoracic hemangioma was non-amenable to surgical and radiological interventions. Vincristine was initiated after 2week trial of Prednisolone for persistent thrombocytopenia and non-regressing haemangioma. Over a week the lesion shrunk with improving platelet count. Vincristine was stopped after 4weeks in view of no further tumour shrinkage. The patient was discharged on steroid advised for 6-12 months and showed significant tumour regression at 3month.
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References
Abass K, Saad H, Kherala M, Abd-Elsayed AA. Successful treatment of kasabach-merritt syndrome with vincristine and surgery: a case report and review of literature. Cases J. 2008 May 23;1(1):9. doi: https://doi.org/10.1186/1757-1626-1-9.
Hall GW. Kasabach-Merritt syndrome: pathogenesis and management. Br J Haematol. 2001 Mar;112(4):851-62.
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 Aug;15(4):295–305.
Hesselmann S, Micke O, Marquardt T, Baas S, Bramswig JH, Harms E, et al. Case report: Kasabach-Merritt syndrome: a review of the therapeutic options and a case report of successful treatment with radiotherapy and interferon alpha. Br J Radiol. 2002 Feb;75(890):180–4.
Enjolras O, Mulliken JB, Wassef M, Frieden IJ, Rieu PN, Burrows PE, et al. Residual lesions after Kasabach-Merritt phenomenon in 41 patients. J Am Acad Dermatol. 2000 Feb;42(2 Pt 1):225-35.
Haisley-Royster C, Enjolras O, Frieden IJ, Garzon M, Lee M, Oranje A, et al. Kasabach-merritt phenomenon: a retrospective study of treatment with vincristine. J Pediatr Hematol Oncol. 2002 Aug-Sep;24(6):459-62.
Shin HY, Ryu KH, Ahn HS. Stepwise multimodal approach in the treatment of Kasabach-Merritt syndrome. Pediatr Int. 2000 Dec;42(6):620-4.
Wananukul S, Nuchprayoon I, Seksarn P. Treatment of Kasabach-Merritt syndrome: a stepwise regimen of prednisolone, dipyridamole, and interferon. Int J Dermatol. 2003 Sep;42(9):741-8.
Mitsuhashi N, Furuta M, Sakurai H, Takahashi T, Kato S, Nozaki M, et al. Outcome of radiation therapy for patients with Kasabach-Merritt syndrome. Int J Radiat Oncol Biol Phys. 1997 Sep 1;39(2):467-73.
Akyüz C, Emir S, Büyükpamukçu M, Büyükpamukçu N, Cağlar M, Kale G, et al. Successful treatment with interferon alfa in infiltrating angiolipoma: a case presenting with Kasabach-Merritt syndrome. Arch Dis Child. 2003 Jan;88(1):67–8.
Drolet BA, Scott LA, Esterly NB, Gosain AK. Early surgical intervention in a patient with Kasabach-Merritt phenomenon. J Pediatr. 2001 May;138(5):756–8.
Pasqual E, Bacchetti S, Gasparini D, Sponza M, Cagol PP. Embolisation of arteriovenous intrahepatic fistulas associated with diffuse haemangiomatosis of the liver. Report of a case in an adult and review of the literature. Chir Ital. 2007 Oct;59(5):701–5.
Maguiness S, Guenther L. Kasabach-merritt syndrome. J Cutan Med Surg. 2002 Jul-Aug;6(4):335-9. Epub 2002 Apr 15.
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