Kasabach-Merritt Phenomenon: Classic Presentation and Management Options

Overview

Journal Clin Med Insights Blood Disord

Publisher Sage Publications

Specialty Hematology

Date 2017 Jun 6

PMID 28579853

Citations 40

Authors

Priya Mahajan

Judith Margolin

Ionela Iacobas

Affiliations

Soon will be listed here.

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.

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References

Fernandez-Pineda I, Lopez-Gutierrez J, 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(8):641-5. DOI: 10.3109/08880018.2010.508299. View

Drolet B, Trenor 3rd C, Brandao L, Chiu Y, Chun R, Dasgupta R . Consensus-derived practice standards plan for complicated Kaposiform hemangioendothelioma. J Pediatr. 2013; 163(1):285-91. DOI: 10.1016/j.jpeds.2013.03.080. View

Lopez V, Marti N, Pereda C, Martin J, Ramon D, Mayordomo E . Successful management of Kaposiform hemangioendothelioma with Kasabach-Merritt phenomenon using vincristine and ticlopidine. Pediatr Dermatol. 2009; 26(3):365-6. DOI: 10.1111/j.1525-1470.2009.00923.x. View

Moimeaux V, Taieb A, Legrain V, Meraud J, Jimenez M, Choussat A . Aspirin-ticlopidin in Kasabach-Merritt syndrome. Lancet. 1992; 340(8810):55. DOI: 10.1016/0140-6736(92)92470-z. View

Chang E, Boyd A, Nelson C, Crowley D, Law T, Keough K . Successful treatment of infantile hemangiomas with interferon-alpha-2b. J Pediatr Hematol Oncol. 1997; 19(3):237-44. DOI: 10.1097/00043426-199705000-00011. View

Sarkar M, Mulliken J, Kozakewich H, Robertson R, Burrows P . Thrombocytopenic coagulopathy (Kasabach-Merritt phenomenon) is associated with Kaposiform hemangioendothelioma and not with common infantile hemangioma. Plast Reconstr Surg. 1997; 100(6):1377-86. DOI: 10.1097/00006534-199711000-00001. View

Ryan C, Price V, John P, Mahant S, Baruchel S, Brandao L . Kasabach-Merritt phenomenon: a single centre experience. Eur J Haematol. 2009; 84(2):97-104. DOI: 10.1111/j.1600-0609.2009.01370.x. View

Tlougan B, Lee M, Drolet B, Frieden I, Adams D, Garzon M . Medical management of tumors associated with Kasabach-Merritt phenomenon: an expert survey. J Pediatr Hematol Oncol. 2013; 35(8):618-22. DOI: 10.1097/MPH.0b013e318298ae9e. View

Zukerberg L, Nickoloff B, Weiss S . Kaposiform hemangioendothelioma of infancy and childhood. An aggressive neoplasm associated with Kasabach-Merritt syndrome and lymphangiomatosis. Am J Surg Pathol. 1993; 17(4):321-8. DOI: 10.1097/00000478-199304000-00001. View

10.

Mitsuhashi N, Furuta M, Sakurai H, Takahashi T, Kato S, Nozaki M . Outcome of radiation therapy for patients with Kasabach-Merritt syndrome. Int J Radiat Oncol Biol Phys. 1997; 39(2):467-73. DOI: 10.1016/s0360-3016(97)00140-5. View

11.

Rodriguez V, Lee A, Witman P, Anderson P . Kasabach-merritt phenomenon: case series and retrospective review of the mayo clinic experience. J Pediatr Hematol Oncol. 2009; 31(7):522-6. DOI: 10.1097/MPH.0b013e3181a71830. View

12.

Shin H, Ryu K, Ahn H . Stepwise multimodal approach in the treatment of Kasabach-Merritt syndrome. Pediatr Int. 2001; 42(6):620-4. DOI: 10.1046/j.1442-200x.2000.01302.x. View

13.

Mulliken J, Anupindi S, Ezekowitz R, Mihm Jr M . Case records of the Massachusetts General Hospital. Weekly clinicopathological exercises. Case 13-2004. A newborn girl with a large cutaneous lesion, thrombocytopenia, and anemia. N Engl J Med. 2004; 350(17):1764-75. DOI: 10.1056/NEJMcpc049002. View

14.

STRAUB P, Kessler S, Schreiber A, FRICK P . Chronic intravascular coagulation in Kasabach-Merritt syndrome. Preferential accumulation of fibrinogen 131 I in a giant hemangioma. Arch Intern Med. 1972; 129(3):475-8. View

15.

Hermans D, van Beynum I, van der Vijver R, Kool L, de Blaauw I, van der Vleuten C . Kaposiform hemangioendothelioma with Kasabach-Merritt syndrome: a new indication for propranolol treatment. J Pediatr Hematol Oncol. 2011; 33(4):e171-3. DOI: 10.1097/MPH.0b013e3182152e4e. View

16.

Lyons L, North P, Lai F, Stoler M, Folpe A, Weiss S . Kaposiform hemangioendothelioma: a study of 33 cases emphasizing its pathologic, immunophenotypic, and biologic uniqueness from juvenile hemangioma. Am J Surg Pathol. 2004; 28(5):559-68. DOI: 10.1097/00000478-200405000-00001. View

17.

Haisley-Royster C, Enjolras O, Frieden I, Garzon M, Lee M, Oranje A . Kasabach-merritt phenomenon: a retrospective study of treatment with vincristine. J Pediatr Hematol Oncol. 2002; 24(6):459-62. DOI: 10.1097/00043426-200208000-00010. View

18.

Koerper M, Addiego Jr J, Delorimier A, Lipow H, Price D, Lubin B . Use of aspirin and dipyridamole in children with platelet trapping syndromes. J Pediatr. 1983; 102(2):311-4. DOI: 10.1016/s0022-3476(83)80550-2. View

19.

Deb G, Jenkner A, De Sio L, Boldrini R, Bosman C, Standoli N . Spindle cell (Kaposiform) hemangioendothelioma with Kasabach-Merritt syndrome in an infant: successful treatment with alpha-2A interferon. Med Pediatr Oncol. 1997; 28(5):358-61. DOI: 10.1002/(sici)1096-911x(199705)28:5<358::aid-mpo6>3.0.co;2-g. View

20.

Enjolras O, Soupre V, Picard A . Uncommon benign infantile vascular tumors. Adv Dermatol. 2009; 24:105-24. DOI: 10.1016/j.yadr.2008.09.008. View