Kasabach-Merritt Syndrome Clinical Presentation

  • Author: Bernice R Krafchik, MBChB, FRCPC; Chief Editor: Emmanuel C Besa, MD   more...
 
Updated: Feb 22, 2010
 

History

The typical patient with Kasabach-Merritt phenomenon is an infant or young child, often male, who initially presents with a reddish brown or blue, tender skin lesion that rapidly evolves into a violaceous, bulging mass with petechiae and purpura. There is often a history of a blue lesion on the skin before the swelling and bruising. Occasionally there may be purpura and petechiae seen in many areas over the body with no history of a previous vascular lesion. This is seen in retroperitoneal and mediastinal lesions with DIC.

The natural history of kaposiform hemangioendothelioma 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 kaposiform endotheliomas develop into Kasabach-Merritt phenomenon.

Next

Physical

  • Lesions of kaposiform hemangioendothelioma or tufted angioma or a mixture of both present with a blue or reddish brown discoloration and induration on the skin.
    • When the thrombocytopenia increases, a large violaceous, ecchymotic indurated mass forms.
    • 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 they can occur.
  • Bleeding from thrombocytopenia and coagulopathy is observed both locally and at times distantly (DIC).
Previous
Next

Causes

Kasabach-Merritt phenomenon occurs in lesions of kaposiform hemangioendotheliomas and tufted angiomas; the etiology of the phenomenon is unknown but may follow trauma in the area of the kaposiform endothelioma.

Previous
Proceed to Differential Diagnoses
 
 
Contributor Information and Disclosures
Author

Bernice R Krafchik, MBChB, FRCPC  Professor Emeritus, Department of Pediatrics, Section of Dermatology, University of Toronto

Bernice R Krafchik, MBChB, FRCPC is a member of the following medical societies: American Academy of Dermatology, American Dermatological Association, Canadian Medical Association, College of Physicians and Surgeons of Ontario, Royal College of Physicians and Surgeons of Canada, and Society for Pediatric Dermatology

Disclosure: Nothing to disclose.

Coauthor(s)

Linda K Hendricks, MD  Assistant Professor, Department of Internal Medicine, Section of Hematology and Oncology, Mercer University School of Medicine

Linda K Hendricks, MD is a member of the following medical societies: American Society of Hematology

Disclosure: Nothing to disclose.

Guy B Faguet, MD  Former Professor, Department of Medicine, Section of Hematology and Oncology, Medical College of Georgia

Guy B Faguet, MD is a member of the following medical societies: American Association of Immunologists, American Society of Hematology, International Society of Hematology, New York Academy of Sciences, Southern Medical Association, and Southern Society for Clinical Investigation

Disclosure: Nothing to disclose.

Sejal Kuthiala, MD  Staff Physician, Department of Internal Medicine, Medical College of Georgia

Sejal Kuthiala, MD is a member of the following medical societies: American Medical Association

Disclosure: Nothing to disclose.

Specialty Editor Board

Michael Paul Kosty, MD  Associate Director, Associate Professor, Department of Internal Medicine, Divisions of Supportive Care Services and Hematology and Oncology, Ida M and Cecil H Green Cancer Center, Scripps Clinic

Michael Paul Kosty, MD is a member of the following medical societies: American College of Physicians, American Society of Hematology, and Phi Beta Kappa

Disclosure: Nothing to disclose.

Francisco Talavera, PharmD, PhD  Senior Pharmacy Editor, eMedicine

Disclosure: eMedicine Salary Employment

Ronald A Sacher, MB, BCh, MD, FRCPC  Professor, Internal Medicine and Pathology, Director, Hoxworth Blood Center, University of Cincinnati Academic Health Center

Ronald A Sacher, MB, BCh, MD, FRCPC is a member of the following medical societies: American Association for the Advancement of Science, American Association of Blood Banks, American Clinical and Climatological Association, American Society for Clinical Pathology, American Society of Hematology, College of American Pathologists, International Society of Blood Transfusion, International Society on Thrombosis and Haemostasis, and Royal College of Physicians and Surgeons of Canada

Disclosure: Glaxo Smith Kline Honoraria Speaking and teaching; Talecris Honoraria Board membership

Rajalaxmi McKenna, MD, FACP  Southwest Medical Consultants, SC, Department of Medicine, Good Samaritan Hospital, Advocate Health Systems

Rajalaxmi McKenna, MD, FACP is a member of the following medical societies: American Society of Clinical Oncology, American Society of Hematology, and International Society on Thrombosis and Haemostasis

Disclosure: Nothing to disclose.

Chief Editor

Emmanuel C Besa, MD  Professor, Department of Medicine, Division of Hematologic Malignancies, Kimmel Cancer Center, Thomas Jefferson University

Emmanuel C Besa, MD is a member of the following medical societies: American Association for Cancer Education, American College of Clinical Pharmacology, American Federation for Medical Research, American Society of Clinical Oncology, American Society of Hematology, and New York Academy of Sciences

Disclosure: Nothing to disclose.

References

  1. Sadan N, Wolach B. Treatment of hemangiomas of infants with high doses of prednisone. J Pediatr. Jan 1996;128(1):141-6. [Medline].

  2. Nako Y, Fukushima N, Igarashi T, et al. Successful interferon therapy in a neonate with life-threatening Kasabach-Merritt syndrome. J Perinatol. May-Jun 1997;17(3):244-7. [Medline].

  3. 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. Nov-Dec 1998;20(6):567-9. [Medline].

  4. Wananukul S, Nuchprayoon I, Seksarn P. Treatment of Kasabach-Merritt syndrome: a stepwise regimen of prednisolone, dipyridamole, and interferon. Int J Dermatol. Sep 2003;42(9):741-8. [Medline].

  5. 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. May-Jun 2009;13(3):155-9. [Medline].

  6. Lopez V, Marti N, Pereda C, et al. Successful management of kaposiform hemangioendothelioma with Kasabach-Merritt phenomenon using vincristine and ticlopidine. Pediatr Dermatol. May-Jun 2009;26(3):365-6. [Medline].

  7. Hara K, Yoshida T, Kajiume T, et al. Successful treatment of Kasabach-Merritt syndrome with vincristine and diagnosis of the hemangioma using three-dimensional imaging. Pediatr Hematol Oncol. Jul-Aug 2009;26(5):375-80. [Medline].

  8. 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. Sep 2009;31(9):690-2. [Medline].

  9. Yoon HS, Lee JH, Moon HN, et al. Successful treatment of retroperitoneal infantile hemangioendothelioma with Kasabach-Merritt syndrome using steroid, alpha-interferon, and vincristine. J Pediatr Hematol Oncol. Dec 2009;31(12):952-4. [Medline].

  10. Mitsuhashi N, Furuta M, Sakurai H, et al. Outcome of radiation therapy for patients with Kasabach-Merritt syndrome. Int J Radiat Oncol Biol Phys. Sep 1 1997;39(2):467-73. [Medline].

  11. Blei F, Karp N, Rofsky N, et al. Successful multimodal therapy for kaposiform hemangioendothelioma complicated by Kasabach-Merritt phenomenon: case report and review of the literature. Pediatr Hematol Oncol. Jul-Aug 1998;15(4):295-305. [Medline].

  12. Leong E, Bydder S. Use of radiotherapy to treat life-threatening Kasabach-Merritt syndrome. J Med Imaging Radiat Oncol. Feb 2009;53(1):87-91. [Medline].

  13. Hosono S, Ohno T, Kimoto H, 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].

  14. Komiyama M, Nakajima H, Kitano S, et al. Endovascular treatment of huge cervicofacial hemangioma complicated by Kasabach-Merritt syndrome. Pediatr Neurosurg. Jul 2000;33(1):26-30. [Medline].

  15. 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. Nov 2009;4(5):453-7. [Medline].

  16. Haisley-Royster C, Enjolras O, Frieden IJ, et al. Kasabach-Merritt phenomenon: a retrospective study of treatment with vincristine. J Pediatr Hematol Oncol. Aug-Sep 2002;24(6):459-62. [Medline].

  17. Alvarez-Mendoza A, Lourdes TS, Ridaura-Sanz C, Ruiz-Maldonado R. Histopathology of vascular lesions found in Kasabach-Merritt syndrome: review based on 13 cases. Pediatr Dev Pathol. Nov-Dec 2000;3(6):556-60. [Medline].

  18. Arnaout MK, Pappo AS. Kaposiform hemangioendothelioma with associated Kasabach-Merritt phenomenon. The Hemophilia Growth and Development Study. J Pediatr. Dec 1998;133(6):788. [Medline].

  19. Biban P. Kasabach-Merritt syndrome and interferon alpha: still a controversial issue. Arch Dis Child. Jul 2003;88(7):645-6. [Medline].

  20. Brasanac D, Janic D, Boricic I, et al. Retroperitoneal kaposiform hemangioendothelioma with tufted angioma-like features in an infant with Kasabach-Merritt syndrome. Pathol Int. Sep 2003;53(9):627-31. [Medline].

  21. Enjolras O, Mulliken JB, Wassef M, et al. Residual lesions after Kasabach-Merritt phenomenon in 41 patients. J Am Acad Dermatol. Feb 2000;42(2 pt 1):225-35. [Medline].

  22. Enjolras O, Wassef M, Mazoyer E, et al. Infants with Kasabach-Merritt syndrome do not have "true" hemangiomas. J Pediatr. Apr 1997;130(4):631-40. [Medline].

  23. Frieden IJ, Eichenfield LF, Esterly NB, et al. Guidelines of care for hemangiomas of infancy. American Academy of Dermatology Guidelines/Outcomes Committee. J Am Acad Dermatol. Oct 1997;37(4):631-7. [Medline].

  24. Martinez AE, Robinson MJ, Alexis JB. Kaposiform hemangioendothelioma associated with nonimmune fetal hydrops. Arch Pathol Lab Med. Jun 2004;128(6):678-81. [Medline].

  25. Mazoyer E, Enjolras O, Laurian C, Houdart E, Drouet L. Coagulation abnormalities associated with extensive venous malformations of the limbs: differentiation from Kasabach-Merritt syndrome. Clin Lab Haematol. Aug 2002;24(4):243-51. [Medline].

  26. Mwanda OW, Wanjohi EW, Cheserem EN. Kasabach Merritt syndrome in an adult female African: case report. East Afr Med J. Mar 1990;67(3):214-20. [Medline].

  27. Shulkin BL, Argenta LC, Cho KJ, Castle VP. Kasabach-Merritt syndrome: treatment with epsilon-aminocaproic acid and assessment by indium 111 platelet scintigraphy. J Pediatr. Nov 1990;117(5):746-9. [Medline].

  28. Velin P, Dupont D, Golkar A, Valla JS. Neonatal Kasabach-Merritt syndrome healed by complete surgical excision of the angioma. Arch Pediatr. Mar 1998;5(3):295-7. [Medline].

  29. Vin-Christian K, McCalmont TH, Frieden IJ. Kaposiform hemangioendothelioma. An aggressive, locally invasive vascular tumor that can mimic hemangioma of infancy. Arch Dermatol. Dec 1997;133(12):1573-8. [Medline].

 
Previous
Next
 
Leg with a Kaposiform hemangioendothelioma, lesion associated with Kasabach-Merritt Syndrome.
Back of an arm showing the typical bruising associated with Kasabach-Merritt Syndrome.
 
 
 
All material on this website is protected by copyright, Copyright © 1994-2012 by WebMD LLC.
This website also contains material copyrighted by 3rd parties.

DISCLAIMER: The content of this Website is not influenced by sponsors. The site is designed primarily for use by qualified physicians and other medical professionals. The information contained herein should NOT be used as a substitute for the advice of an appropriately qualified and licensed physician or other health care provider. The information provided here is for educational and informational purposes only. In no way should it be considered as offering medical advice. Please check with a physician if you suspect you are ill.