Kasabach-Merritt Syndrome Clinical Presentation
- Author: Alexandra C Cheerva, MD, MS; Chief Editor: Hassan M Yaish, MD more...
Visible cutaneous blue, violaceous, or reddish-brown lesions are often the presenting features in patients with Kasabach-Merritt syndrome (KMS). Most lesions are located on the extremities. Some infants and older children with visceral lesions present with an enlarged abdomen. Those with hepatic kaposiform hemangioendotheliomas also may present with hepatomegaly or jaundice. These vascular lesions may continue to enlarge during the first 18 months of life.
The thrombocytopenia and consumptive coagulopathy associated with KMS may not initially be severe. However, symptoms may worsen as the lesion enlarges and the infant grows. Affected infants may present soon after birth or may not come to medical attention for several months. Affected individuals rarely present as late as the second or third decade of life.
Petechiae, bruising, and frank bleeding may be the initial symptoms prompting medical treatment. The lesions may be painful.
The large volume of blood circulating through the lesion may cause high-output congestive heart failure in infants. Cardiovascular compromise or collapse, petechiae, and bleeding may resemble acute overwhelming sepsis. When no cutaneous lesion is present, the physician must search for vascular lesions located in a visceral organ (eg, the spleen, liver, or brain).
Some patients with diffuse cavernous kaposiform hemangioendothelioma (KHE) of a visceral organ may present with anemia, thrombocytopenia, coagulopathy, and bleeding, which may be misdiagnosed as immune thrombocytopenic purpura.
The natural history of KHE is that of slow regression, with the lesion leaving a reddish-brown discoloration that often does not resolve completely. It is unknown what percentage of KHE lesions develop into KMS.
A cutaneous vascular lesion is usually obvious, often appearing as a large irregular bruise (see the first image below). These lesions may occur anywhere on the body and may grow through the first 12-18 months of life, often circumscribed by widespread, overlying, shiny and dusky, purple skin. Lesions of KHE (see the second image below), tufted angioma (TA), or a mixture of both present with a blue or reddish-brown discoloration and skin induration. When thrombocytopenia increases, a large violaceous, ecchymotic indurated mass forms.
Affected infants may exhibit petechiae, bruising, and bleeding. Bruising and ecchymoses may occur at distant sites. Internal lesions may present with only bruising and ecchymoses on the skin. The lesions are usually painful and tender. Aggressive infiltration with ulceration and infection is rare but can occur. Bleeding from thrombocytopenia and coagulopathy is observed both locally and, at times, distantly (ie, disseminated intravascular coagulation [DIC]).
Physical signs of high-output cardiac failure include tachycardia, feeding difficulty, and shock. Pallor may be evident in patients with significant anemia.
The clinical course of KMS is unpredictable, and effective treatment depends on control of the invasive tumor before secondary complications occur.
Various complications of KMS relate to the site of the vascular lesion. For example, hemangiomas of the chest that invade the thorax can compromise lung expansion and cause respiratory insufficiency.
Other complications that may be seen include the following:
Severe thrombocytopenia (platelet count < 5 X 10 9/L (< 5000/µL)
Ulceration and bleeding into the vascular lesion
Bleeding secondary to DIC and unresponsive to platelet transfusions (potentially fatal)
Toxicity from the agents used to treat KMS (eg, secondary malignancy from radiation therapy)
High-output cardiac failure (potentially fatal)
Infections from skin breakdown with sepsis
Kasabach HH, Merritt KK. Capillary hemangioma with extensive purpura: report of a case. Am J Dis Child. 1940. 59:1063-70.
Kelly M. Kasabach-Merritt phenomenon. Pediatr Clin North Am. 2010 Oct. 57(5):1085-9. [Medline].
Hatley RM, Sabio H, Howell CG, Flickinger F, Parrish RA. Successful management of an infant with a giant hemangioma of the retroperitoneum and Kasabach-Merritt syndrome with alpha-interferon. J Pediatr Surg. 1993 Oct. 28(10):1356-7; discussion 1358-9. [Medline].
Martin MC, Harrington H, Wong WW. Massive congenital kaposiform hemangioendothelioma of the eyelid in a neonate. J Craniofac Surg. 2011 Nov. 22(6):e38-41. [Medline].
Enjolras O, Wassef M, Mazoyer E, et al. Infants with Kasabach-Merritt syndrome do not have "true" hemangiomas. J Pediatr. 1997 Apr. 130(4):631-40. [Medline].
Szlachetka DM. Kasabach-Merritt syndrome: a case review. Neonatal Netw. 1998 Feb. 17(1):7-15. [Medline].
Payne LG, Hayward CPM, Kelton JG. Destruction of red cells by the vasculature and reticuloendothelial system. Hematology of Infancy and Childhood. 1998. 523-43.
Berman B, Lim H. Concurrent cutaneous and hepatic hemangiomata in infancy: report of a case and a review of the literature. J Dermatol Surg Oncol. 1978 Nov. 4(11):869-73. [Medline].
Ram SP. Kasabach-Merritt syndrome and Down's syndrome. J R Soc Med. 1997 Mar. 90(3):159-60. [Medline].
Enjolras O, Riche MC, Merland JJ, Escande JP. Management of alarming hemangiomas in infancy: a review of 25 cases. Pediatrics. 1990 Apr. 85(4):491-8. [Medline].
Tang JY, Chen J, Pan C, Yin MZ, Zhu M. Diffuse cavernous hemangioma of the spleen with Kasabach-Merritt syndrome misdiagnosed as idiopathic thrombocytopenia in a child. World J Pediatr. 2008 Aug. 4(3):227-30. [Medline].
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 Sep 1. 39(2):467-73. [Medline].
Pampin C, Devillers A, Treguier C, et al. Intratumoral consumption of indium-111-labeled platelets in a child with splenic hemangioma and thrombocytopenia. J Pediatr Hematol Oncol. 2000 May-Jun. 22(3):256-8. [Medline].
Sadan N, Wolach B. Treatment of hemangiomas of infants with high doses of prednisone. J Pediatr. 1996 Jan. 128(1):141-6. [Medline].
Nako Y, Fukushima N, Igarashi T, Hoshino M, Sugiyama M, Tomomasa T, et al. Successful interferon therapy in a neonate with life-threatening Kasabach-Merritt syndrome. J Perinatol. 1997 May-Jun. 17(3):244-7. [Medline].
Hu B, Lachman R, Phillips J, et al. Kasabach-Merritt syndrome-associated kaposiform hemangioendothelioma successfully treated with cyclophosphamide, vincristine, and actinomycin D. J Pediatr Hematol Oncol. 1998 Nov-Dec. 20(6):567-9. [Medline].
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. [Medline].
Drucker AM, Pope E, Mahant S, Weinstein M. Vincristine and corticosteroids as first-line treatment of Kasabach-Merritt syndrome in kaposiform hemangioendothelioma. J Cutan Med Surg. 2009 May-Jun. 13(3):155-9. [Medline].
López V, Martí N, Pereda C, Martín JM, Ramón D, Mayordomo E, et al. Successful management of Kaposiform hemangioendothelioma with Kasabach-Merritt phenomenon using vincristine and ticlopidine. Pediatr Dermatol. 2009 May-Jun. 26(3):365-6. [Medline].
Hara K, Yoshida T, Kajiume T, Ohno N, Kawaguchi H, Kobayashi M. Successful treatment of Kasabach-Merritt syndrome with vincristine and diagnosis of the hemangioma using three-dimensional imaging. Pediatr Hematol Oncol. 2009 Jul-Aug. 26(5):375-80. [Medline].
Hartman KR, Moncur JT, Minniti CP, Creamer KM. Mediastinal Kaposiform hemangioendothelioma and Kasabach-Merritt phenomenon in an infant: treatment with interferon. J Pediatr Hematol Oncol. 2009 Sep. 31(9):690-2. [Medline].
Yoon HS, Lee JH, Moon HN, Seo JJ, Im HJ, Goo HW. Successful treatment of retroperitoneal infantile hemangioendothelioma with Kasabach-Merritt syndrome using steroid, alpha-interferon, and vincristine. J Pediatr Hematol Oncol. 2009 Dec. 31(12):952-4. [Medline].
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 Jul-Aug. 15(4):295-305. [Medline].
Leong E, Bydder S. Use of radiotherapy to treat life-threatening Kasabach-Merritt syndrome. J Med Imaging Radiat Oncol. 2009 Feb. 53(1):87-91. [Medline].
Argenta LC, Bishop E, Cho KJ, et al. Complete resolution of life-threatening hemangioma by embolization and corticosteroids. Plast Reconstr Surg. 1982 Dec. 70(6):739-44. [Medline].
Stanley P, Gomperts E, Woolley MM. Kasabach-Merritt syndrome treated by therapeutic embolization with polyvinyl alcohol. Am J Pediatr Hematol Oncol. 1986 Winter. 8(4):308-11. [Medline].
Jiang RS, Hu R. Successful treatment of Kasabach-Merritt syndrome arising from kaposiform hemangioendothelioma by systemic corticosteroid therapy and surgery. Int J Clin Oncol. 2012 Oct. 17(5):512-6. [Medline].
Akyuz C, Emir S, Buyukpamukcu M, 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. [Medline]. [Full Text].
Ezekowitz RA, Mulliken JB, Folkman J. Interferon alfa-2a therapy for life-threatening hemangiomas of infancy. N Engl J Med. 1992 May 28. 326(22):1456-63. [Medline].
Michaud AP, Bauman NM, Burke DK, et al. Spastic diplegia and other motor disturbances in infants receiving interferon-alpha. Laryngoscope. 2004 Jul. 114(7):1231-6. [Medline].
de la Hunt MN. Kasabach-Merritt syndrome: dangers of interferon and successful treatment with pentoxifylline. J Pediatr Surg. 2006 Jan. 41(1):e29-31. [Medline].
Haisley-Royster C, Enjolras O, Frieden IJ, et al. Kasabach-merritt phenomenon: a retrospective study of treatment with vincristine. J Pediatr Hematol Oncol. 2002 Aug-Sep. 24(6):459-62. [Medline].
Hurvitz SA, Hurvitz CH, Sloninsky L, Sanford MC. Successful treatment with cyclophosphamide of life-threatening diffuse hemangiomatosis involving the liver. J Pediatr Hematol Oncol. 2000 Nov-Dec. 22(6):527-32. [Medline].
Perez Payarols J, Pardo Masferrer J, Gomez Bellvert C. Treatment of life-threatening infantile hemangiomas with vincristine. N Engl J Med. 1995 Jul 6. 333(1):69. [Medline].
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 Nov. 27(8):641-5. [Medline].
Traivaree C, Lumkul R, Torcharus K, Krutuecho T, Sriphaisal T. Outcome of Kasabach-Merritt phenomenon: the role of vincristine as monotherapy: report of a case. J Med Assoc Thai. 2012 May. 95 Suppl 5:S181-5. [Medline].
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 Apr. 54(2):285-7. [Medline].
Leaute-Labreze C, Dumas de la Roque E, Hubiche T, Boralevi F, Thambo JB, Taieb A. Propranolol for severe hemangiomas of infancy. N Engl J Med. 2008 Jun 12. 358(24):2649-51. [Medline].
Su L, Wang D, Fan X. Comprehensive therapy for hemangioma presenting with Kasabach-Merritt syndrome in the maxillofacial region. J Oral Maxillofac Surg. 2015 Jan. 73 (1):92-8. [Medline].
George M, Singhal V, Sharma V, Nopper AJ. Successful surgical excision of a complex vascular lesion in an infant with Kasabach-Merritt syndrome. Pediatr Dermatol. 2002 Jul-Aug. 19(4):340-4. [Medline].
Yang YH, Lee PI, Lin KH, Tsang YM. Absolute ethanol embolotherapy for hemangioma with Kasabach-Merritt syndrome. Zhonghua Min Guo Xiao Er Ke Yi Xue Hui Za Zhi. 1998 Jan-Feb. 39(1):51-4. [Medline].
Hosono S, Ohno T, Kimoto H, Nagoshi R, Shimizu M, Nozawa M, et al. Successful transcutaneous arterial embolization of a giant hemangioma associated with high-output cardiac failure and Kasabach-Merritt syndrome in a neonate: a case report. J Perinat Med. 1999. 27(5):399-403. [Medline].
Komiyama M, Nakajima H, Kitano S, Sakamoto H, Kurimasa H, Ozaki H. Endovascular treatment of huge cervicofacial hemangioma complicated by Kasabach-Merritt syndrome. Pediatr Neurosurg. 2000 Jul. 33(1):26-30. [Medline].
Wolfe SQ, Farhat H, Elhammady MS, Moftakhar R, Aziz-Sultan MA. Transarterial embolization of a scalp hemangioma presenting with Kasabach-Merritt syndrome. J Neurosurg Pediatr. 2009 Nov. 4(5):453-7. [Medline].
Enomoto Y, Yoshimura S, Egashira Y, Iwama T. Transarterial embolization for cervical hemangioma associated with Kasabach-merritt syndrome. Neurol Med Chir (Tokyo). 2011. 51(5):375-8. [Medline].
Poetke M, Philipp C, Berlien HP. Flashlamp-pumped pulsed dye laser for hemangiomas in infancy: treatment of superficial vs mixed hemangiomas. Arch Dermatol. 2000 May. 136(5):628-32. [Medline].
Aylett SE, Williams AF, Bevan DH, Holmes SJ. The Kasabach-Merritt syndrome: treatment with intermittent pneumatic compression. Arch Dis Child. 1990 Jul. 65(7):790-1. [Medline].
Schild SE, Buskirk SJ, Frick LM, Cupps RE. Radiotherapy for large symptomatic hemangiomas. Int J Radiat Oncol Biol Phys. 1991 Aug. 21(3):729-35. [Medline].
Hesselmann S, Micke O, Marquardt T, 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. [Medline].