Kasabach-Merritt Phenomenon: Classic Presentation and Management Options

Priya Mahajan, Judith Margolin, Ionela Iacobas, Priya Mahajan, Judith Margolin, Ionela Iacobas

Abstract

Kasabach-Merritt phenomenon (KMP) is a rare consumptive coagulopathy associated with specific vascular tumors, kaposiform hemangioendothelioma, and tufted angioma. Kasabach-Merritt phenomenon, characterized by profound thrombocytopenia, hypofibrinogenemia, elevated fibrin split products, and rapid tumor growth, can be life-threatening. Severe symptomatic anemia may also be present. With prompt diagnosis and management, KMP can resolve and vascular tumors have been shown to regress. This review highlights the clinical presentation, histopathology, management, and treatment of KMP associated with kaposiform hemangioendothelioma, and less frequently tufted angioma. A classic clinical case is described to illustrate the presentation and our management of a patient with KMP.

Keywords: Kasabach-Merritt phenomenon (KMP); coagulopathy; kaposiform hemangioendothelioma (KHE); sirolimus; thrombocytopenia.

Conflict of interest statement

DECLARATION OF CONFLICTING INTEREST: The author(s) declared no potential conflicts of interest with respect to the research, authorship, and/or publication of this article.

Figures

Figure 1.
Figure 1.
Classic clinical case of Kaposiform hemangioendothelioma with Kasabach-Merritt phenomenon (KMP) diagnosed in a 5-week-old patient. Diagnosis, response to blood product transfusion, and resolution of KMP with sirolimus + steroids therapy. (A) Platelet counts (normal: 150-450 × 103/µL), (B) hemoglobin (normal: 9.5-13.5 g/dL), (C) fibrinogen (normal: 220-440 mg/dL), and (D) d-dimer (normal: <0.4 µg/mL).
Figure 2.
Figure 2.
Classic clinical case of Kaposiform hemangioendothelioma with Kasabach-Merritt phenomenon (KMP) diagnosed in a 5-week-old patient. Extension of cutaneous involvement. Photos presented with mother’s permission. (A) At the peak of activity of KMP (day 4)—patient with purpura, edema, irregular margins, and extensive size. (B) After 3 weeks of treatment, the cutaneous component improved significantly. (C) After 2 months of therapy with sirolimus and steroids, complete resolution of the cutaneous component was achieved.

References

    1. Kasabach HH, Merritt KK. Capillary hemangioma with extensive purpura: report of a case. Am J Dis Child. 1940;59:1063–1070.
    1. Rodriguez V, Lee A, Witman PM, Anderson PA. Kasabach-Merritt phenomenon: case series and retrospective review of the mayo clinic experience. J Pediatr Hematol Oncol. 2009;31:522–526.
    1. Sarkar M, Mulliken JB, Kozakewich HP, Robertson RL, Burrows PE. Thrombocytopenic coagulopathy (Kasabach-Merritt phenomenon) is associated with kaposiform hemangioendothelioma and not with common infantile hemangioma. Plast Reconstr Surg. 1997;100:1377–1386.
    1. Enjolras O, Wassef M, Mazoyer E, et al. Infants with Kasabach-Merritt syndrome do not have “true” hemangiomas. J Pediatr. 1997;130:631–640.
    1. Croteau SE, Liang MG, Kozakewich HP, et al. Kaposiform hemangioendothelioma: atypical features and risks of Kasabach-Merritt phenomenon in 107 referrals. J Pediatr. 2013;162:142–147.
    1. Lyons LL, North PE, Lai FM-M, Stoler MH, Folpe AL, Weiss SW. Kaposiform hemangioendothelioma: a study of 33 cases emphasizing its pathologic, immunophenotypic, and biologic uniqueness from juvenile hemangioma. Am J Surg Pathol. 2004;28:559–568.
    1. Zukerberg LR, Nickoloff BJ, Weiss SW. Kaposiform hemangioendothelioma of infancy and childhood. An aggressive neoplasm associated with Kasabach-Merritt syndrome and lymphangiomatosis. Am J Surg Pathol. 1993;17:321–328.
    1. Le Huu AR, Jokinen CH, Ruben BP, et al. Expression of prox1, lymphatic endothelial nuclear transcription factor, in Kaposiform hemangioendothelioma and tufted angioma. Am J Surg Pathol. 2010;34:1563–1573.
    1. Enjolras O, Soupre V, Picard A. Uncommon benign infantile vascular tumors. Adv Dermatol. 2008;24:105–124.
    1. Seo SK, Suh JC, Na GY, Kim IS, Sohn KR. Kasabach-Merritt syndrome: identification of platelet trapping in a tufted angioma by immunohistochemistry technique using monoclonal antibody to CD61. Pediatr Dermatol. 1999;16:392–394.
    1. Straub PW, Kessler S, Schreiber A, Frick PG. Chronic intravascular coagulation in Kasabach-Merritt syndrome. Preferential accumulation of fibrinogen 131 I in a giant hemangioma. Arch Intern Med. 1972;129:475–478.
    1. Phillips WG, Marsden JR. Kasabach-Merritt syndrome exacerbated by platelet transfusion. J R Soc Med. 1993;86:231–232.
    1. Mulliken JB, Anupindi S, Ezekowitz RAB, Mihm MC., Jr. Case 13-2004: a newborn girl with a large cutaneous lesion, thrombocytopenia, and anemia. N Engl J Med. 2004;350:1764–1775.
    1. Koerper MA, Addiego JE, de Lorimier AA, Lipow H, Price D, Lubin BH. Use of aspirin and dipyridamole in children with platelet trapping syndromes. J Pediatr. 1983;102:311–314.
    1. Drolet BA, Scott LA, Esterly NB, Gosain AK. Early surgical intervention in a patient with Kasabach-Merritt phenomenon. J Pediatr. 2001;138:756–758.
    1. Mitsuhashi N, Furuta M, Sakurai H, et al. Outcome of radiation therapy for patients with Kasabach-Merritt syndrome. Int J Radiat Oncol Biol Phys. 1997;39:467–473.
    1. Malhotra Y, Yang CS, McNamara J, Antaya RJ. Congenital kaposiform hemangioendothelioma with Kasabach-Merritt phenomenon successfully treated with low-dose radiation therapy. Pediatr Dermatol. 2014;31:595–598.
    1. Shin HY, Ryu KH, Ahn HS. Stepwise multimodal approach in the treatment of Kasabach-Merritt syndrome. Pediatr Int. 2000;42:620–624.
    1. Garcia-Monaco R, Giachetti A, Peralta O, et al. Kaposiform hemangioendothelioma with Kasabach-Merritt phenomenon: successful treatment with embolization and vincristine in two newborns. J Vasc Interv Radiol. 2012;23:417–422.
    1. Haisley-Royster C, Enjolras O, Frieden IJ, et al. Kasabach-Merritt phenomenon: a retrospective study of treatment with vincristine. J Pediatr Hematol Oncol. 2002;24:459–462.
    1. Wang Z, Li K, Yao W, Dong K, Xiao X, Zheng S. Steroid-resistant kaposiform hemangioendothelioma: a retrospective study of 37 patients treated with vincristine and long-term follow-up. Pediatr Blood Cancer. 2015;62:577–580.
    1. Deb G, Jenkner A, De Sio L, et al. Spindle cell (Kaposiform) hemangioendothelioma with Kasabach-Merritt syndrome in an infant: successful treatment with alpha-2A interferon. Med Pediatr Oncol. 1997;28:358–361.
    1. Chang E, Boyd A, Nelson CC, et al. Successful treatment of infantile hemangiomas with interferon-alpha-2b. J Pediatr Hematol Oncol. 1997;19:237–244.
    1. Chiu YE, Drolet BA, Blei F, et al. Variable response to propranolol treatment of kaposiform hemangioendothelioma, tufted angioma, and Kasabach-Merritt phenomenon. Pediatr Blood Cancer. 2012;59:934–938.
    1. Hermans DJJ, van Beynum IM, van der Vijver RJ, Kool LJS, de Blaauw I, van der Vleuten CJM. Kaposiform hemangioendothelioma with Kasabach-Merritt syndrome: a new indication for propranolol treatment. J Pediatr Hematol Oncol. 2011;33:e171–e173.
    1. Ryan C, Price V, John P, et al. Kasabach-Merritt phenomenon: a single centre experience. Eur J Haematol. 2010;84:97–104.
    1. Drolet BA, Trenor CC, III, Brandão LR, et al. Consensus-derived practice standards plan for complicated Kaposiform hemangioendothelioma. J Pediatr. 2013;163:285–291.
    1. Tlougan BE, Lee MT, Drolet BA, Frieden IJ, Adams DM, Garzon MC. Medical management of tumors associated with Kasabach-Merritt phenomenon: an expert survey. J Pediatr Hematol Oncol. 2013;35:618–622.
    1. Adams DM, Trenor CC, Hammill AM, et al. Efficacy and safety of sirolimus in the treatment of complicated vascular anomalies. Pediatrics. 2016;137:e20153257.
    1. Fernandez-Pineda I, Lopez-Gutierrez JC, Ramirez G, Marquez C. Vincristine-ticlopidine-aspirin: an effective therapy in children with Kasabach-Merritt phenomenon associated with vascular tumors. Pediatr Hematol Oncol. 2010;27:641–645.
    1. Fernandez-Pineda I, Lopez-Gutierrez JC, Chocarro G, Bernabeu-Wittel J, Ramirez-Villar GL. Long-term outcome of vincristine-aspirin-ticlopidine (VAT) therapy for vascular tumors associated with Kasabach-Merritt phenomenon. Pediatr Blood Cancer. 2013;60:1478–1481.
    1. Moimeaux V, Taieb A, Legrain V, et al. Aspirin-ticlopidin in Kasabach-Merritt syndrome. Lancet. 1992;340:55.
    1. Lopez V, Marti N, Pereda C, et al. Successful management of Kaposiform hemangioendothelioma with Kasabach-Merritt phenomenon using vincristine and ticlopidine. Pediatr Dermatol. 2009;26:365–366.

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