Kasabach-Merritt Syndrome Medication

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

Medication Summary

No single modality is favored over others, and many regimens have been investigated, with variable success. Corticosteroids are the drugs most commonly used, although often with poor results. In addition to high-dose oral steroids, pulsed or intravenous steroids have also been used.

Some success with interferon alfa-2a (3 million U/m2/d or 3 times/wk) has been reported, although the failure rate is high. The subcutaneous injection may result in nausea, fever, and/or neutropenia. Some infants have developed spastic diplegia after receiving interferon alfa.

Case reports have described the administration of vincristine to treat Kasabach-Merritt phenomenon.[3, 5, 6, 7, 9, 16] This treatment is used with increasing frequency. Nevertheless there have been treatment failures.

The hematologic agents epsilon aminocaproic acid, aspirin, dipyridamole or ticlopidine, pentoxifylline, cryoprecipitate, and heparin have all been used with various efficacies.

Radiation therapy has been abandoned because of its lack of success and late sequelae of cancers in the treated area.

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Corticosteroids

Class Summary

Systemic corticosteroids are synthetic chemicals that have been used to successfully treat proliferative hemangiomas: the results with Kasabach-Merritt phenomenon are usually not as successful. High-dose oral prednisone 2-4 mg/kg/d can rapidly increase the platelet count.

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Prednisone (Deltasone, Orasone, Meticorten)

 

May increase sensitivity of arterioles and precapillaries to vasoconstrict. May competitively inhibit other hormonal agents.

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Triamcinolone (Amcort)

 

For inflammation responsive to steroids; decreases inflammation by suppressing migration of polymorphonuclear leukocytes and reversing capillary permeability.

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Antineoplastic Agents, Vesicant

Class Summary

These agents inhibit cell growth and proliferation. The standard dose is 1-1.5 mg/m2 or 0.05-0.065 mg/kg once weekly.

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Vincristine (Oncovin, Vincasar PFS)

 

Mechanism of action is uncertain; may involve a decrease in reticuloendothelial cell function or an increase in platelet production.

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Interferons

Class Summary

These substances are naturally produced proteins that have antiviral, antitumoral, and immunomodulatory actions. Alfa, beta, and gamma interferons may be administered topically, systemically, or intralesionally.

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Interferon alfa-2a (Roferon A)

 

Protein product manufactured by recombinant DNA technology. Mechanism of antitumoral activity not clearly understood; however, direct antiproliferative effects against malignant cells and modulation of host immune response may be important.

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Interferon alfa-2b (Intron A)

 

Protein product manufactured by recombinant DNA technology. Mechanism of antitumor activity is not clearly understood; however, direct antiproliferative effects against malignant cells and modulation of host immune response may play important roles.

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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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