Pediatric Kasabach-Merritt Syndrome Clinical Presentation

  • Author: Alexandra C Cheerva, MD, MS; Chief Editor: Max J Coppes, MD, PhD, MBA   more...
 
Updated: Mar 1, 2012
 

History

  • Visible cutaneous giant hemangioma or multiple smaller hemangiomas are often the presenting features in patients with Kasabach-Merritt syndrome (KMS).[10] 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.[11] 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.[12]
  • Lesions may be painful.
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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.[13]
  • 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.
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Causes

  • The underlying cause of large cutaneous or visceral hemangiomas is unknown.
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Contributor Information and Disclosures
Author

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

Alexandra C Cheerva, MD, MS 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 School of Medicine

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; Editor-in-Chief, Medscape Drug Reference

Disclosure: Nothing to disclose.

Gary D Crouch, MD  Associate Professor, Program Director of Pediatric Hematology-Oncology Fellowship, Department of Pediatrics, 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 SI Chan, MBBS, FRCP(C), FAAP  Associate Senior Scientist, Research Institute; Professor, Division of Hematology/Oncology, Department of Pediatrics, The Hospital for Sick Children, University of Toronto Faculty of Medicine, Canada

Helen SI 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, Center for Cancer and Blood Disorders, Children's National Medical Center; Professor of Medicine, Oncology, and Pediatrics, Georgetown University School of Medicine; Clinical Professor of Pediatrics, George Washington University School of Medicine and Health Sciences

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.

Additional Contributors

The authors and editors of eMedicine gratefully acknowledge the contributions of previous author Carlos Suarez, MD, to the development and writing of this article.

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