eMedicine Specialties > Hematology > Red Blood Cells and Disorders

Kasabach-Merritt Syndrome

Author: Bernice R Krafchik, MBChB, FRCPC, Professor Emeritus, Department of Pediatrics, Section of Dermatology, University of Toronto
Coauthor(s): Linda K Hendricks, MD, Assistant Professor, Department of Internal Medicine, Section of Hematology and Oncology, Mercer University School of Medicine; Guy B Faguet, MD, Former Professor, Department of Medicine, Section of Hematology and Oncology, Medical College of Georgia; Sejal Kuthiala, MD, Staff Physician, Department of Internal Medicine, Medical College of Georgia
Contributor Information and Disclosures

Updated: Jun 8, 2007

Introduction

Background

In 1940, Kasabach and Merritt described a male infant with a discolored, indurated lesion on his left thigh that rapidly grew and affected the entire left leg, scrotum, abdomen, and thorax. In addition, the infant also had consumptive thrombocytopenia. This association has become known as Kasabach-Merritt syndrome (KMS). The original case is now known to have been associated with kaposiform hemangioendothelioma and thrombocytopenia.

Pathophysiology

The vascular lesion triggers (1) intravascular coagulation with platelet trapping and activation and (2) consumption of coagulation factors.

Thrombocytopenia is a consumptive process in the vascular tumor that is corrected when the tumor is controlled. The platelets consumed in the process, with the release of platelet-derived growth factor, which is a mitogen. Increased thrombin generation occurs, followed by the development of disseminated intravascular coagulopathy (DIC). Thrombin (also a mitogen) and other cytokines are released with DIC.

Frequency

United States

KMS is uncommon.

Mortality/Morbidity

KMS can be lethal; the estimated overall mortality rate ranges from 10-37%. Morbidity and mortality are associated with visceral involvement (particularly in the retroperitoneum and mediastinum), hemorrhage related to aggressive invasion, profound thrombocytopenia, severe infections, and iatrogenic complications.

Race

No racial or ethnic predilection is known.

Sex

Boys and men may be affected slightly more often than girls and women.

Age

KMS typically occurs in newborns or early infancy, though prenatal cases (diagnosed with the aid of ultrasonography) and rare adult cases have been reported.

Clinical

History

The typical patient with Kasabach-Merritt syndrome (KMS) is an infant, often male, who initially presents with a reddish brown skin lesion that evolves into a violaceous, bulging mass.

The condition is often diagnosed in utero by using fetal ultrasonography. Tenderness is common, and ulceration may occur. Coagulopathy may cause petechiae or ecchymosis in any area, and bleeding may occur.

The natural history of kaposiform hemangioendothelioma is that of slow regression, with the lesion leaving a reddish brown discoloration. Large lesions do not become completely involuted.

Physical

  • Lesions have reddish brown discoloration with induration.
    • As the thrombocytopenia increases, a large violaceous, ecchymotic indurated mass forms.
    • Bruising usually occurs in other areas as well.
  • Kaposi hemangioendothelioma, tufted angiomas, or a mixture of both are the skin lesions associated with KMS.
    • These lesions can occur anywhere on the skin which is the organ most commonly affected.
    • Lesions of the internal organs often cause bruising on the skin.
  • Lesions are usually painful and tender.
  • Aggressive infiltration with ulceration, and infection is rare but can occur.
  • Bleeding from thrombocytopenia and coagulopathy is frequently observed.

Causes

KMS occurs in lesions of kaposiform hemangioendotheliomas and tufted angiomas, but the etiology 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
Multimedia: Kasabach-Merritt Syndrome
References
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References

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Further Reading

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Keywords

KMS, Kaposi hemangioendothelioma, Kaposi's hemangioendothelioma, kaposiform hemangioendothelioma, consumptive thrombocytopenia, thrombocytopenic purpura, tufted angiomas, vascular lesions, ecchymotic lesions, interferon alfa, Kasabach Merritt syndrome, vascular tumor, mitogen

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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 Clinical Oncology and 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.

Medical Editor

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 Clinical Oncology, American Society of Hematology, and Phi Beta Kappa
Disclosure: Nothing to disclose.

Pharmacy Editor

Francisco Talavera, PharmD, PhD, Senior Pharmacy Editor, eMedicine
Disclosure: Nothing to disclose.

Managing Editor

Ronald A Sacher, MD, Director of the Hoxworth Blood Center, Professor, Departments of Internal Medicine and Pathology, University of Cincinnati Medical Center
Ronald A Sacher, MD is a member of the following medical societies: American Society of Hematology
Disclosure: Glaxo Smith Kline Honoraria Speaking and teaching; Talecris Honoraria Board membership

CME Editor

Rajalaxmi McKenna, MD, FACP, Consulting Staff, Department of Medicine, Southwest Medical Consultants, SC, 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.

 
 
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