Kasabach-Merritt Syndrome Workup

  • Author: Bernice R Krafchik, MBChB, FRCPC; Chief Editor: Emmanuel C Besa, MD   more...
 
Updated: Feb 22, 2010
 

Laboratory Studies

  • Complete blood cell (CBC) count - To check for thrombocytopenia
  • Fibrinogen level - To check for decreased fibrinogen levels
  • Fibrin split products - To check for elevation
  • D-dimers - To check for elevation
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Imaging Studies

  • Magnetic resonance imaging (MRI) or computed tomography (CT) scanning reveals a vascular enhancing mass that is difficult to differentiate from a vascular malformation, solid tumor, or proliferative vascular lesion.
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Other Tests

Clinicians must monitor blood work, with special emphasis on the platelet count

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Procedures

It sometimes is necessary to do CT scans and MRIs. Interventional radiologists are at times involved in eliminating vessels and closing off lesions.

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Histologic Findings

Enjolras and colleagues characterized the histology of kaposiform hemangioendothelioma and tufted angioma that are the vascular lesions associated with Kasabach-Merritt phenomenon. The original case report described lobules of fine capillaries separated by cellular intercapillary tissue consisting of spindle-shaped cells.

The histologic picture of kaposiform hemangioendothelioma consists of lobules or sheets of tightly packed spindle cells or rounded endothelial cells and pericytes. The cellular areas have an infiltrative pattern in the dermis, subcutaneous fat, and muscles and generally contain few obvious vascular lumina.

Tufted angiomas are composed of small tufts or lobules of rounded capillaries with small lumina. The tufts are discrete and evenly distributed in a cannonball pattern and are characterized by peripheral, crescentic, slitlike vessels and an associated fibrosis.

Both kaposiform hemangioendothelioma and tufted angioma contain aggregates of rounded, dilated capillaries lined by attenuated endothelial cells with small, dark nuclei and filled with red blood cells. Microthrombi and hemosiderin deposits are often present. Lymphlike vessels are often part of the lesion. Findings of both kaposiform hemangioendothelioma and tufted angioma often appear in the same patient and are thought to be variations of one another.

The histology of classic hemangioma of infancy, which is the most common benign vascular neoplasm in children, is distinct from these proliferations and is not associated with Kasabach-Merritt phenomenon.

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Contributor Information and Disclosures
Author

Bernice R Krafchik, MBChB, FRCPC  Professor Emeritus, Department of Pediatrics, Section of Dermatology, University of Toronto

Bernice R Krafchik, MBChB, FRCPC is a member of the following medical societies: American Academy of Dermatology, American Dermatological Association, Canadian Medical Association, College of Physicians and Surgeons of Ontario, Royal College of Physicians and Surgeons of Canada, and Society for Pediatric Dermatology

Disclosure: Nothing to disclose.

Coauthor(s)

Linda K Hendricks, MD  Assistant Professor, Department of Internal Medicine, Section of Hematology and Oncology, Mercer University School of Medicine

Linda K Hendricks, MD is a member of the following medical societies: American Society of Hematology

Disclosure: Nothing to disclose.

Guy B Faguet, MD  Former Professor, Department of Medicine, Section of Hematology and Oncology, Medical College of Georgia

Guy B Faguet, MD is a member of the following medical societies: American Association of Immunologists, American Society of Hematology, International Society of Hematology, New York Academy of Sciences, Southern Medical Association, and Southern Society for Clinical Investigation

Disclosure: Nothing to disclose.

Sejal Kuthiala, MD  Staff Physician, Department of Internal Medicine, Medical College of Georgia

Sejal Kuthiala, MD is a member of the following medical societies: American Medical Association

Disclosure: Nothing to disclose.

Specialty Editor Board

Michael Paul Kosty, MD  Associate Director, Associate Professor, Department of Internal Medicine, Divisions of Supportive Care Services and Hematology and Oncology, Ida M and Cecil H Green Cancer Center, Scripps Clinic

Michael Paul Kosty, MD is a member of the following medical societies: American College of Physicians, American Society of Hematology, and Phi Beta Kappa

Disclosure: Nothing to disclose.

Francisco Talavera, PharmD, PhD  Senior Pharmacy Editor, eMedicine

Disclosure: eMedicine Salary Employment

Ronald A Sacher, MB, BCh, MD, FRCPC  Professor, Internal Medicine and Pathology, Director, Hoxworth Blood Center, University of Cincinnati Academic Health Center

Ronald A Sacher, MB, BCh, MD, FRCPC is a member of the following medical societies: American Association for the Advancement of Science, American Association of Blood Banks, American Clinical and Climatological Association, American Society for Clinical Pathology, American Society of Hematology, College of American Pathologists, International Society of Blood Transfusion, International Society on Thrombosis and Haemostasis, and Royal College of Physicians and Surgeons of Canada

Disclosure: Glaxo Smith Kline Honoraria Speaking and teaching; Talecris Honoraria Board membership

Rajalaxmi McKenna, MD, FACP  Southwest Medical Consultants, SC, Department of Medicine, Good Samaritan Hospital, Advocate Health Systems

Rajalaxmi McKenna, MD, FACP is a member of the following medical societies: American Society of Clinical Oncology, American Society of Hematology, and International Society on Thrombosis and Haemostasis

Disclosure: Nothing to disclose.

Chief Editor

Emmanuel C Besa, MD  Professor, Department of Medicine, Division of Hematologic Malignancies, Kimmel Cancer Center, Thomas Jefferson University

Emmanuel C Besa, MD is a member of the following medical societies: American Association for Cancer Education, American College of Clinical Pharmacology, American Federation for Medical Research, American Society of Clinical Oncology, American Society of Hematology, and New York Academy of Sciences

Disclosure: Nothing to disclose.

References

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Leg with a Kaposiform hemangioendothelioma, lesion associated with Kasabach-Merritt Syndrome.
Back of an arm showing the typical bruising associated with Kasabach-Merritt Syndrome.
 
 
 
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