Pediatric Kasabach-Merritt Syndrome 

  • Author: Alexandra C Cheerva, MD; Chief Editor: Max J Coppes, MD, PhD, MBA   more...
 
Updated: Feb 23, 2009
 

Background

Hemangiomas, the most common tumors of infancy, typically undergo rapid postnatal growth for several months, followed by a prolonged phase of involution. The combination of hemangioma, thrombocytopenia, and coagulopathy is termed Kasabach-Merritt syndrome (KMS).[1] The hemangioma may be an obvious superficial lesion or a lesion within a visceral organ or even within the brain. Thrombocytopenia is often severe (ie, < 50,000 platelets/mcL). Thrombocytopenia and consumptive coagulopathy are not complications of all hemangiomas, and size alone does not determine which hemangiomas are associated with thrombocytopenia and coagulopathies. Kasabach-Merritt syndrome is an infrequent but potentially fatal complication of rapidly growing hemangiomas in infants.[2]

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Pathophysiology

The thrombocytopenia associated with hemangiomas is caused by a localized consumptive coagulopathy. The vascular lesion causes platelet trapping and activation, with consumption of coagulation factors. The activation of platelets also promotes further growth of vascular tissue.

The cause of the lesions associated with Kasabach-Merritt syndrome is unknown, and whether the underlying lesion in Kasabach-Merritt syndrome is a single anatomic entity or a heterogenous group of entities is unclear. Several types of vascular tumors have been associated with Kasabach-Merritt syndrome (eg, capillary and cavernous hemangiomas, infantile hemangioendothelioma, Kaposiform hemangioendothelioma [KHE], lymphangioma, tufted angioma [TA]).[3] Kasabach-Merritt syndrome is associated more often with KHE and TA vascular tumors than with common hemangiomas.[4]

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Epidemiology

Frequency

United States

Hemangiomas are common vascular tumors that occur in as many as 2.5% of neonates. Most are benign, and 70-80% regress by age 7 years. Some hemangiomas are life threatening; one hemangioma in 300 is associated with coagulopathy.[5]

Mortality/Morbidity

Mortality and morbidity rates are influenced by the anatomic location, depth, and extent of the hemangioma. Other causes of morbidity and mortality include infections and organ toxicity, usually as a result of therapy. Bleeding, which is secondary to the consumptive coagulopathy, is the primary cause of death. Untreated Kasabach-Merritt syndrome has a 10-37% mortality rate.[5]

Hemolytic anemia resulting from physical damage to the RBCs may be mild, moderate, or severe. Direct antibody test (DAT) or Coombs test results are negative in patients with anemia, and anemia is secondary to microangiopathic destruction of the RBCs.[6]

Heart failure often occurs in affected infants as a result of the large volume of blood flowing through the giant hemangioma.[7]

Race

Kasabach-Merritt syndrome has no racial predilection.

Sex

Incidence is slightly increased in females.

Age

Although infants younger than 1 year are most commonly affected, older children and adults have developed Kasabach-Merritt syndrome. Include Kasabach-Merritt syndrome in the differential diagnosis of chronic thrombocytopenia at any age.

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Contributor Information and Disclosures
Author

Alexandra C Cheerva, MD  Associate Professor of Pediatrics, Hematology/Oncology Division, Director of Pediatric Blood and Marrow Transplantation, University of Louisville; Attending Staff, Section of Pediatric Hematology and Oncology, Kosair Children's Hospital

Alexandra C Cheerva, MD is a member of the following medical societies: American Society for Blood and Marrow Transplantation, American Society of Clinical Oncology, American Society of Pediatric Hematology/Oncology, Children's Oncology Group, International Pediatric Transplant Association, and Kentucky Medical Association

Disclosure: Enzon Corporation Honoraria Speaking and teaching

Coauthor(s)

Salvatore Bertolone, MD  Director, Division of Pediatric Hematology/Oncology, Department of Pediatrics, Kosair Children's Hospital; Professor, University of Louisville School of Medicine

Salvatore Bertolone, MD is a member of the following medical societies: American Academy of Pediatrics, American Association for Cancer Education, American Association of Blood Banks, American Cancer Society, American Society of Hematology, American Society of Pediatric Hematology/Oncology, and Kentucky Medical Association

Disclosure: Nothing to disclose.

Ashok B Raj, MD  Associate Professor, Section of Pediatric Hematology and Oncology, Department of Pediatrics, Kosair Children's Hospital, University of Louisville

Ashok B Raj, MD is a member of the following medical societies: American Academy of Pediatrics, American Society of Pediatric Hematology/Oncology, Children's Oncology Group, and Kentucky Medical Association

Disclosure: Nothing to disclose.

Specialty Editor Board

Gary R Jones, MD  Associate Medical Director, Clinical Development, Berlex Laboratories

Gary R Jones, MD is a member of the following medical societies: American Academy of Pediatrics, American Society of Pediatric Hematology/Oncology, and Western Society for Pediatric Research

Disclosure: Nothing to disclose.

Mary L Windle, PharmD  Adjunct Associate Professor, University of Nebraska Medical Center College of Pharmacy; Pharmacy Editor, eMedicine

Disclosure: Nothing to disclose.

Gary D Crouch, MD  Program Director of Pediatric Hematology-Oncology Fellowship, Department of Pediatrics, Associate Professor, Uniformed Services University of the Health Sciences

Gary D Crouch, MD is a member of the following medical societies: American Academy of Pediatrics and American Society of Hematology

Disclosure: Nothing to disclose.

Helen SL Chan, MBBS, FRCP(C), FAAP  Senior Scientist, Research Institute; Professor, Division of Hematology/Oncology, Department of Pediatrics, The Hospital for Sick Children, University of Toronto, Canada

Helen SL Chan, MBBS, FRCP(C), FAAP is a member of the following medical societies: American Academy of Pediatrics, American Association for Cancer Research, American Society of Hematology, and Royal College of Physicians and Surgeons of Canada

Disclosure: Nothing to disclose.

Chief Editor

Max J Coppes, MD, PhD, MBA  Senior Vice President, Children's National Medical Center (Center for Cancer and Blood Disorders); Director, Center for Cancer and Immunology Research, Children's Research Institute, Children's National Medical Center; Professor of Medicine, Oncology, and Pediatrics, Georgetown University

Max J Coppes, MD, PhD, MBA is a member of the following medical societies: American Association for Cancer Research, American Society of Pediatric Hematology/Oncology, and Society for Pediatric Research

Disclosure: Nothing to disclose.

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