EC Paediatrics

Introduction: Kasabach-Merritt syndrome is a rare form of vascular tumor that are often misdiagnosed, which is complicated by intra-tumor coagulopathy and requiring urgent management.

Objective: This review highlights the clinical presentation, histopathology, management, and treatment of Kasabach-Merritt syndrome. A clinical case compiled in the pediatric department of the university Hospital Center of Oran Algeria, is described to illustrate the presentation and our management of a patient with Kasabach-Merritt syndrome.

Observation: A female infant was admitted, on the thirtieth day of life, for a purplish red swelling of the left hemiface. He had hemolytic anemia, profound thrombocytopenia and consumptive coagulopathy. It was successfully managed with a combination of high dose of oral corticosteroids and vincristine.

Conclusion: Vincristine and oral corticosteroids were useful for our patient and may be considered an option for first-line therapy of Kasabach-Merritt syndrome. The absence of a codified therapeutic protocol makes the management of this tumor a real challenge.

Keywords: Kasabach-Merritt Syndrome; Thrombocytopenia; Hemangiomas; Coagulopathy; Vascular Tumor; Kaposiform Hemangioendothelioma

1. Minard-Colin V., et al. “Les tumeurs mésenchymateuses du nouveau-né - Soft tissue tumors in neonates”. Archives de Pédiatrie7 (2009): 1039-1048.
2. ISSVA Classification of Vascular Anomalies ©2018 International Society for the Study of Vascular Anomalies .Available at "issva.org/classification" (2022).
3. Enjolras O., et al. “Infants with Kasabach-Merritt syndrome do not have "true" hemangiomas”. The Journal of Pediatrics4 (1997): 631-640.
4. Alvarez-Mendoza A., et al. “Histopathology of vascular lesions found in Kasabach-Merritt syndrome: review based on 13 cases”. Pediatric and Developmental Pathology6 (2000): 556-560.
5. Drolet BA., et al. “Consensus-derived practice standards plan for complicated Kaposiform hemangioendothelioma”. The Journal of Pediatrics1 (2013): 285-291.
6. Kasabach HH and Merritt KK. “Capillary hemangioma with extensive purpura: report of a case”. American Journal of Diseases of Children 59 (1940): 1063-1070.
7. Croteau SE., et al. “Kaposiform hemangioendothelioma: atypical features and risks of Kasabach-Merritt phenomenon in 107 referrals”. The Journal of Pediatrics1 (2013): 142-147.
8. Gözdaşoğlu S. “Kasabach-Merritt Syndrome in an Adult: A Comment”. Turkish Journal of Hematology1 (2019): 52-53.
9. O’Rafferty C., et al. “Recent advances in the pathobiology and management of Kasabach-Merritt phenomenon”. British Journal of Haematology 171 (2015): 38-51.
10. Schmid I., et al. “Kaposiform hemangioendothelioma in children: a benign vascular tumor with multiple treatment options”. World Journal of Pediatrics4 (2018): 322-329.
11. Zukerberg LR., et al. “Kaposiform hemangioendothelioma of infancy and childhood. An aggressive neoplasm associated with Kasabach-Merritt syndrome and lymphangiomatosis”. The American Journal of Surgical Pathology 17 (1993): 321-328.
12. Rodriquez V., et al. “Kasabach-Merritt phenomenon: case series and retrospective review of the mayo clinic experience”. Journal of Pediatric Hematology/Oncology7 (2009): 522-526.
13. Sarkar M., et al. “Thrombocytopenic coagulopathy (Kasabach-Merritt phenomenon) is associated with Kaposiform hemangioendothelioma and not with common infantile hemangioma”. Plastic and Reconstructive Surgery6 (1997): 1377-1386.
14. Mahajan P., et al. “Kasabach-Merritt Phenomenon: Classic Presentation and Management Options”. Clinical Medicine Insights: Blood Disorders 10 (2017): 1179545X17699849.
15. Ryan C., et al. “Kasabach-Merritt phenomenon: a single centre experience”. The European Journal of Haematology2 (2010): 97-104.
16. Kelly M. “Kasabach-Merritt phenomenon”. Pediatric Clinics of North America5 (2010): 1085-1089.
17. Hall GW. “Kasabach-Merritt syndrome: Pathogenesis and management”. British Journal of Haematology 112 (2001): 851-862.
18. O’Rafferty C., et al. “Recent advances in the pathobiology and management of Kasabach-Merritt phenomenon”. British Journal of Haematology 171 (2015): 38-51.

19. Lyons LL., et al. “Kaposiform hemangioendothelioma: a study of 33 cases emphasizing its pathologic, immunophenotypic, and biologic uniqueness from juvenile Hemangioma”. The American Journal of Surgical Pathology5 (2004): 559-568.
20. Le Huu AR., et al. “Expression of prox1, lymphatic endothelial nuclear transcription factor, in Kaposiform hemangioendothelioma and tufted angioma”. The American Journal of Surgical Pathology 34 (2010): 1563-1573.
21. Enjolras O., et al. “Uncommon benign infantile vascular tumors”. Advances in Dermatology 24 (2008): 105-124.
22. Verheul HM., et al. “Treatment of the Kasabach-Merritt syndrome with pegylated recombinant human megakaryocyte growth and development factor in mice: elevated platelet counts, prolonged survival, and tumor growth inhibition”. Pediatric Research5 (1999): 562-565.
23. Phillips WG and Marsden JR. “Kasabach-Merritt syndrome exacerbated by platelet transfusion”. Journal of the Royal Society of Medicine 86 (1993): 231-232.
24. Pampin C., et al. “Intratumoral consumption of indium-111- labeled platelets in a child with splenic hemangioma and thrombocytopenia”. Journal of Pediatric Hematology/Oncology3 (2000): 256-258.
25. Seon KS., et al. “Kasabach-Merritt syndrome: identification of platelet trapping in a tufted angioma by immunohistochemistry technique using monoclonal antibody to CD61”. Pediatric Dermatology5 (1999): 392-394.
26. Ortel T., et al. “Antifibrinolytic therapy in the management of the kasabach merritt syndrome”. American Journal of Hematology1 (1988): 44-48.
27. Larsen E., et al. “Kasabach-Merritt syndrome: therapeutic considerations”. Pediatrics 79 (1987): 971-980.
28. Adams DM. “Special considerations in vascular anomalies: hematologic management”. Clinics in Plastic Surgery 38 (2011): 153-160.
29. Zhou SY., et al. “Successful treatment of Kasabach-Merritt syndrome with transarterial embolization and corticosteroids”. Journal of Pediatric Surgery3 (2013): 673-676.
30. Wananukul S., et al. “Treatment of Kasabaeh-Merritt syndrome: a stepwise regimen of prednisolone, dipyridamole, and interferon”. International Journal of Dermatology 42 (2003) :741-748.
31. Perez Payarols J., et al. “Treatment of life-threatening infantile hemangiomas with vincristine”. The New England Journal of Medicine 333 (1995): 69-70.
32. Hu B., et al. “Kasabach-Merritt syndrome-associated kaposiform hemangioendothelioma successfully treated with cyclophosphamide, vincristine, and actinomycin”. Pediatric Hematology Oncology Journal6 (1998): 567-569.
33. Chiu YE., et al. “Variable response to propranolol treatment of kaposiform hemangioendothelioma, tufted angioma, and Kasabach-Merritt phenomenon”. Pediatric Blood and Cancer 59 (2012): 934-938.
34. Hermans DJJ., et al. “Kaposiform hemangioendothelioma with Kasabach-Merritt syndrome: a new indication for propranolol treatment”. Journal of Pediatric Hematology/Oncology 33 (2011): e171-e173.
35. Shin HY., et al. “Stepwise multimodal approach in the treatment of Kasabach-Merritt syndrome”. Pediatrics International 42 (2000): 620-624.
36. Deb G., et al. “Spindle cell (Kaposiform) hemangioendothelioma with Kasabach-Merritt syndrome in an infant: successful treatment with alpha-2A interferon”. Medical and Pediatric Oncology 28 (1997): 358-361.
37. Barlow C., et al. “Spastic diplegia as a complication of interferon Alfa-2a treatment of hemangiomas of infancy”. The Journal of Pediatrics3 (1998): 527-530.
38. Haisley-Royster C., et al. “Kasabach-Merritt phenomenon: a retrospective study of treatment with vincristine”. Journal of Pediatric Hematology/Oncology6 (2002): 459-462.
39. Ohtsuka T., et al. “Angioblastoma (Nakagawa) with hyperhidrosis, and relapse after a 10-year interval”. British Journal of Dermatology1 (2000): 223-224.
40. Hauer JGU., et al. “Effective treatment of kaposiform hemangioendotheliomas associated with Kasabach–Merritt phenomenon using four-drug regimen”. Pediatric Blood and Cancer 49 (2007): 852-872.
41. Jiang BH and Liu LZ. “PI3K/PTEN signaling in tumorigenesis and angiogenesis”. Biochimica et Biophysica Acta 1784 (2008): 150-158.
42. Perry B., et al. “AKT1 overexpression in endothelial cells leads to the development of cutaneous vascular malformations in vivo”. Archives of Dermatological Research 143 (2007): 504-506.
43. Morris PN., et al. “Functional analysis of a mutant form of the receptor tyrosine kinase Tie2 causing venous malformations”. Journal of Molecular Medicine 83 (2005): 58-63.
44. Ji Y., et al. “Sirolimus for the treatment of progressive kaposiform hemangioendothelioma: a multicenter retrospective study”. International Journal of Cancer 141 (2017): 848-855.