Pediatric Kasabach-Merritt Syndrome Follow-up

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

Further Inpatient Care

  • Patients with the fulminant form of Kasabach-Merritt syndrome (KMS) require supportive care, including replacement therapy with platelets, fibrinogen concentrate, fresh frozen plasma (FFP), cryoprecipitate and antifibrinolytic drugs, and antiplatelet agents.
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Further Outpatient Care

  • Continue supportive treatments on an outpatient basis.
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Inpatient & Outpatient Medications

  • Continue supportive inpatient treatments.
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Transfer

  • Transfer neonates with giant hemangiomas and disseminated intravascular coagulation (DIC) to a facility with a neonatal ICU (NICU) capable of providing supportive care. Infants who present very early are likely to have a more severe form of Kasabach-Merritt syndrome, and they require extensive intervention.
  • The need to transfer an older child depends on the level of care necessary to support the patient. The general recommendation is to transfer to a tertiary care center all children who require medications other than steroids or who require support using blood products.
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Complications

  • Various complications of Kasabach-Merritt syndrome relate to the site of the hemangioma. For example, hemangiomas of the chest that invade the thorax can compromise lung expansion and cause respiratory insufficiency.
  • Bleeding secondary to DIC and unresponsive to platelet transfusions can cause death.
  • Toxicity can result from the agents used to treat Kasabach-Merritt syndrome (eg, secondary malignancy from radiation therapy).
  • Some patients may experience high-output cardiac failure, which may lead to death.
  • Ulceration and bleeding into the hemangioma can occur in patients with Kasabach-Merritt syndrome.
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Prognosis

  • Patients in whom Kasabach-Merritt syndrome is recognized and properly treated usually have an excellent prognosis because the DIC resolves as the hemangioma recedes and because Kasabach-Merritt syndrome does not recur. Therefore, most children do well if they reach age 2 years.
  • Patients in whom Kasabach-Merritt syndrome is untreated have a 10-37% mortality rate, primarily due to bleeding.
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Patient Education

  • Once the diagnosis of Kasabach-Merritt syndrome is confirmed, inform parents regarding the signs of congestive heart failure and thrombocytopenia (ie, DIC) in infants. These signs include difficulty feeding, petechiae, bruising, hematuria, and bloody stools.
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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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