Kasabach-Merritt Syndrome

Kasabach-Merritt syndrome

141000

HEMANGIOMA-THROMBOCYTOPENIA SYNDROME

Alternative titles; symbols KASABACH-MERRITT SYNDROME; KMS

TEXT

With giant hemangiomas in small children, thrombocytopenia and red cell changes compatible with trauma ('microangiopathic hemolytic anemia') have been observed. The mechanism of the hematologic changes is obscure. No evidence of a simple genetic basis has been discovered. Mulvihill (1982) pointed out to me that hemangioma of the placenta can cause symptomatic thrombocytopenia of the newborn. Experience with the therapy of 6 children with capillary hemangiomas in association with consumptive coagulopathy (Kasabach-Merritt syndrome) was reviewed by Larsen et al. (1987). Sencer et al. (1987) reported the case of a newborn infant with splenic hemangioendothelioma with thrombocytopenia, anemia, and disseminated intravascular coagulation. A splenectomy was indicated despite the known risks of subsequent overwhelming sepsis by encapsulated organisms. Vellodi and Bini (1988) described a severe hyperkalemia resulting in 'malignant ventricular arrhythmias.' They attributed the hyperkalemia to breakdown of erythrocytes. Breakdown of platelets is another possible source.

Enjoiras et al. (1997) concluded that the vascular lesion underlying KMS is not a 'true,' classic, involuting type of hemangioma of infancy. It is a different vascular tumor with a resemblance pathologically to either tufted angioma or kaposiform hemangioendothelioma in association with lymphatic-like vessels.

As indicated, the Kasabach-Merritt syndrome is seen in children with large vascular tumors and is characterized by very low platelet counts and a consumption of coagulation factors causing life-threatening complications. It appears that thrombocytopenia in these patients is caused by intratumoral trapping of platelets. From studies in a mouse model of Kasabach-Merritt syndrome, Verheul et al. (1999) concluded that increased platelet production stimulated by thrombopoietin (THPO; 600044) increased survival and inhibition of tumor growth.

SEE ALSO

Brizel and Raccuglia (1965); David et al. (1983); Propp and Scharfman (1966); Rodriguez-Erdmann et al. (1970)

REFERENCES

1. Brizel, H. E.; Raccuglia, G. :
Giant hemangioma with thrombocytopenia. Radioisotopic demonstration of platelet sequestration. Blood 26: 751-756, 1965.PubMed ID : 5844148

2. David, T. J.; Evans, D. I. K.; Stevens, R. F. :
Haemangioma with thrombocytopenia (Kasabach-Merritt syndrome). Arch. Dis. Child. 58: 1022-1023, 1983.PubMed ID : 6660888

3. Enjoiras, O.; Wassef, M.; Mazoyer, E.; Frieden, I. J.; Rieu, P. N.; Drouet, L.; Taieb, A.; Stalder, J.-F.; Escande, J.-P. :
Infants with Kasabach-Merritt syndrome do not have 'true' hemangiomas. J. Pediat. 130: 631-640, 1997.PubMed ID : 9108863

4. Larsen, E. C.; Zinkham, W. H.; Eggleston, J. C.; Zitelli, B. J. :
Kasabach-Merritt syndrome: therapeutic considerations. Pediatrics 79: 971-980, 1987.PubMed ID : 3108848

5. Mulvihill, J. J. :
Personal Communication. Bethesda, Md., 4/1982.

6. Propp, R. P.; Scharfman, W. B. :
Hemangioma-thrombocytopenia syndrome associated with microangiopathic hemolytic anemia. Blood 28: 623-633, 1966.PubMed ID : 5950934

7. Rodriguez-Erdmann, F.; Murray, J. E.; Moloney, W. C. :
Consumption-coagulopathy in Kasabach-Merritt syndrome. Trans. Assoc. Am. Phys. 83: 168-175, 1970.PubMed ID : 5534090

8. Sencer, S.; Coulter-Knoff, A.; Day, D.; Foker, J.; Thompson, T.; Burke, B. :
Splenic hemangioma with thrombocytopenia in a newborn. Pediatrics 79: 960-966, 1987.PubMed ID : 3588149

9. Vellodi, A.; Bini, R. M. :
Malignant ventricular arrhythmias caused by hyperkalaemia complicating the Kasabach-Merritt syndrome. J. Roy. Soc. Med. 81: 167-168, 1988.

10. Verheul, H. M. W.; Panigrahy, D.; Flynn, E.; Pinedo, H. M.; D'Amato, R. J. :
Treatment of the Kasabach-Merritt syndrome with pegylated recombinant human megakaryocyte growth and development factor in mice: elevated platelet counts, prolonged survival, and tumor growth inhibition. Pediat. Res. 46: 562-565, 1999.PubMed ID : 10541319

CONTRIBUTORS

Victor A. McKusick - updated : 1/10/2000Victor A. McKusick - updated : 6/21/1997

CREATION DATE

Victor A. McKusick : 6/4/1986

EDIT HISTORY

mcapotos : 1/20/2000terry : 1/10/2000terry : 6/24/1997terry : 6/21/1997mimadm : 9/24/1994warfield : 4/8/1994carol : 4/1/1992supermim : 3/16/1992supermim : 3/20/1990ddp : 10/27/1989

Copyright © 1966-2004 Johns Hopkins University

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Kasabach-Merritt syndrome

Hemangioma Thrombocytopenia Syndrome

ImportantIt is possible that the main title of the report Hemangioma Thrombocytopenia Syndrome is not the name you expected. Please check the synonyms listing to find the alternate name(s) and disorder subdivision(s) covered by this report

Synonyms

Kasabach-Merritt Syndrome
Thrombocytopenia-Hemangioma Syndrome

Disorder Subdivisions

None

General Discussion

Hemangioma-Thrombocytopenia Syndrome (also known as Kasabach-Merritt Syndrome) is a rare disorder characterized by an abnormal blood condition in which the low number of blood platelets causes bleeding (thrombocytopenia). The thrombocytopenia is found in association with a benign tumor consisting of large, blood-filled spaces (cavernous hemangioma). The exact cause of this disorder is not known.

Resources

National Organization for Rare Disorders, Inc.55 Kenosia Ave,PO Box 1968Danbury, CT 06813-1968Tel: (203)744-0100 Fax: (203)798-2291 Tel: (800)999-6673TDD: (203)797-9590 Email: orphan@rarediseases.org

NIH/National Heart, Lung and Blood Institute, 31 Center Drive MSC 2480,Building 31A Rm 4A16,Bethesda, MD 20892-2480 Tel: (301)592-8573 Fax: (301)480-4907 Email: nhlbiinfo@rover.nhlbi.nih.gov

Genetic Alliance, 4301 Connecticut Avenue NW,Suite 404,Washington, DC 20008-2304 USA Tel: 2029665557 Fax: 2029668553 Tel: 8003364363 Email: info@geneticalliance.org

Hemangioma Support System c/o Cynthia Schumerth, 1484 Sand Acres Drive, DePere, WI 54115 Tel: (920)336-9399 Hemangioma Newsline PO Box 0358 Findlay, OH 45839-0358 USATel: 4194251593 Fax: 4194251593 Email: hemangnews@msn.com

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