eMedicine Specialties > Pediatrics: General Medicine > Hematology

Kasabach-Merritt Syndrome

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

Updated: Feb 23, 2009

Introduction

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

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

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.

Clinical

History

  • Visible cutaneous giant hemangioma or multiple smaller hemangiomas are often the presenting features in patients with Kasabach-Merritt syndrome (KMS). Most hemangiomas are located on the extremities. Some infants and older children with visceral hemangiomas present with an enlarged abdomen. Those with hepatic hemangiomas also may present with hepatomegaly or jaundice. These hemangiomas may continue to enlarge during the first 18 months of life.
  • The thrombocytopenia and consumptive coagulopathy associated with Kasabach-Merritt syndrome may not initially be initially. However, as the hemangioma enlarges and the infant grows, symptoms may worsen. 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 large volume of blood circulating through the hemangioma may cause high-output congestive heart failure in infants.8 Cardiovascular compromise or collapse, petechiae, and bleeding may resemble acute overwhelming sepsis. When no cutaneous hemangioma is present, the physician must search for hemangiomas located in a visceral organ (eg, spleen, liver, brain).
  • Some patients with diffuse cavernous hemangioma of a visceral organ may present with anemia, thrombocytopenia, coagulopathy and bleeding, which may be misdiagnosed as immune thrombocytopenic purpura.9
  • Lesions may be painful.

Physical

  • A cutaneous hemangioma is usually obvious, often appearing as a large irregular bruise. These hemangiomas may occur anywhere on the body and may grow throughout the first 12-18 months of life. Hemangiomas are often circumscribed by widespread, overlying, shiny and dusky, purple skin.10
  • Physical signs of high-output cardiac failure include tachycardia, feeding difficulty, and shock.
  • Affected infants may exhibit petechiae, bruising, and bleeding.
  • Pallor may be evident in patients with significant anemia.

Causes

  • The underlying cause of large cutaneous or visceral hemangiomas is unknown.

More on Kasabach-Merritt Syndrome

Overview: Kasabach-Merritt Syndrome
Differential Diagnoses & Workup: Kasabach-Merritt Syndrome
Treatment & Medication: Kasabach-Merritt Syndrome
Follow-up: Kasabach-Merritt Syndrome
References
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References

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Further Reading

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Keywords

Kasabach-Merritt syndrome, KMS, giant hemangioma syndrome, thrombocytopenia, giant hemangioma with consumptive coagulopathy, disseminated intravascular coagulation, DIC, neonatal lesion, capillary hemangioma, cavernous hemangioma, infantile hemangioendothelioma, Kaposiform hemangioendothelioma, lymphangioma, tufted angioma, TA, cardiovascular compromise

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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: Nothing to disclose.

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.

Medical Editor

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.

Pharmacy Editor

Mary L Windle, PharmD, Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy, Pharmacy Editor, eMedicine
Disclosure: Pfizer Inc Stock Investment from financial planner; Avanir Pharma Stock Investment from financial planner ; WebMD Salary and stock Employment and investment from financial planner

Managing Editor

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.

CME Editor

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, Executive Director, Center for Cancer and Blood Disorders, 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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