Pediatric Kasabach-Merritt Syndrome Workup

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

Laboratory Studies

  • CBC count with differential, reticulocyte count, platelet count, and peripheral smear are needed to evaluate for microangiopathic hemolytic anemia and thrombocytopenia in Kasabach-Merritt syndrome (KMS).
    • Platelets may be larger than normal when they are released early from the bone marrow.
    • Burr cells and schistocytes may be present in patients with microangiopathic hemolytic anemia.
  • Prothrombin time (PT) and activated partial thromboplastin time (aPTT) are prolonged in patients with significant disseminated intravascular coagulation (DIC).
  • Fibrinogen levels are low in patients with significant DIC.
  • Fibrin degradation products (FDPs) and D-dimer levels are elevated in patients with DIC.
    • A D-dimer test is usually more sensitive than the test for FDPs.
    • Low-grade chronic DIC may be present.
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Imaging Studies

  • Perform appropriate radiographs, CT scans, and MRIs of areas involved with known hemangiomas. These studies are advisable even when all hemangiomas are cutaneous. Scans are important to determine the extent of the visible hemangiomas and to evaluate the patient for possible visceral hemangiomas.
  • If Kasabach-Merritt syndrome is suspected in patients who have no visible hemangiomas, obtain CT scans or MRIs of the head, chest, abdomen, and pelvis to search for visceral hemangiomas.
  • Doppler flow studies may help differentiate a solid mass from a hemangioma.
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Other Tests

  • Perform other tests as needed for clinical evaluation. For example, blood cultures may be appropriate to exclude the possibility of sepsis, and chromosome tests may be needed to exclude certain genetic syndromes.
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Procedures

  • Radionuclide imaging may be indicated.
    • Scans that use chromium isotope 51, indium In 111 oxine–labeled platelets, or iodine I 131–labeled fibrinogen probably are more sensitive than CT scans or MRIs for delineating the size and number of hemangiomas.[14]
    • Radionuclide scans are infrequently used because the diagnosis is usually made clinically. In certain centers, these scans may not be readily available.
    • Strongly consider scintigraphic studies when the etiology of the thrombocytopenia remains uncertain.
  • No procedures are initially needed. If a complete excision cannot be performed, avoid performing a biopsy because it may lead to uncontrolled bleeding at the site.
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Histologic Findings

  • Thrombi containing platelets and fibrin are found within a proliferation of vascular tissue. Varying degrees of fibrosis and necrosis may occur within the lesion.
  • Some authors and physicians have proposed that the term hemangioma be reserved for the proliferative vascular lesions in infants that have a natural history of several months' rapid growth followed by spontaneous regression. This is the typical natural history of the lesions in true Kasabach-Merritt syndrome. Platelet thrombi have been found in microscopic sections of the lesions. Stasis and fibrin thrombi are believed to be central to the pathogenesis of the coagulopathy, with resultant consumption of platelets and coagulation factors, elevated levels of fibrinogen-fibrin degradation products, and microangiopathic fragmentation of RBCs.[15]
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Staging

  • Although formal staging is not usually performed, documenting the extent of the invasion of the hemangioma into normal tissue is important for possible subsequent treatment with surgery or radiation.
  • Hemangiomas do not metastasize.
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Proceed to Treatment & Management
 
 
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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