Kasabach-Merritt Syndrome

Author: Bernice R Krafchik, MBChB, FRCPC; Chief Editor: Emmanuel C Besa, MD more...

Updated: Feb 22, 2010

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Background
Pathophysiology
Epidemiology
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Background

In 1940, Kasabach and Merritt described a male infant with a discolored, indurated lesion on his left thigh that grew rapidly and affected the entire left leg, scrotum, abdomen, and thorax. The infant also had consumptive coagulopathy and thrombocytopenia. This association has become known as Kasabach-Merritt syndrome (KMS) and more recently as the Kasabach-Merritt phenomenon (KMP).

The original case was associated with a kaposiform hemangioendothelioma (not a hemangioma of infancy) and thrombocytopenia. Kasabach-Merritt phenomenon is characterized by profound thrombocytopenia, microangiopathic hemolytic anemia, a consumptive coagulopathy, and an enlarging vascular lesion (either a kaposiform hemangioendothelioma or a tufted angioma, or a mixture of both). It has a mortality rate of up to 30%.

Leg with a Kaposiform hemangioendothelioma, lesion associated with Kasabach-Merritt Syndrome.

Back of an arm showing the typical bruising associated with Kasabach-Merritt Syndrome.

Pathophysiology

The vascular lesion (for unknown reasons) triggers an intravascular coagulation with platelet trapping and consequent thrombocytopenia, and an activation and consumption of coagulation factors. The process is corrected when the tumor is controlled. There is a release of platelet-derived growth factor, increased thrombin, and generation of cytokines, followed by the development of local, and at times, disseminated intravascular coagulopathy (DIC).

Frequency

United States

Kasabach-Merritt phenomenon is uncommon.

International

Reports of Kasabach-Merritt phenomenon are rare.

Mortality/Morbidity

Kasabach-Merritt phenomenon can be lethal; the estimated overall mortality rate ranges from 10% to 37%. Morbidity and mortality are usually associated with visceral involvement (particularly in the retroperitoneal area and mediastinum), hemorrhage related to aggressive invasion, profound thrombocytopenia, DIC, severe infections, and iatrogenic complications.

Race

No racial or ethnic predilection for Kasabach-Merritt phenomenon is known.

Sex

Boys and men are affected slightly more often than girls and women. This is unlike hemangioma of infancy (infantile hemangioma) in which girls are much more affected than boys.

Age

Kasabach-Merritt phenomenon typically occurs in early infancy or childhood, although prenatal cases (diagnosed with the aid of ultrasonography), newborn presentations, and rare adult cases have been reported.

Proceed to Clinical Presentation

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.

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