Kasabach-Merritt syndrome
Synonym:
Hemangioma thrombocytopenia syndrome
Symptoms
Diagnosis
Blood tests which include Complete blood count (CBC) to evaluate anemia and thrombocytopenia; Prothrombin time (PT) to evaluate amount of time necessary for the blood to clot; Fibrinogen level to check for decreased fibrinogen levels; fibrin split products to check for elevation and D-dimers to check for elevation.
CT scans and MRI's can be valuable to determine locations and size of the hemangioma or to detect and unseen tumor.
If lymphedema is present, a lympscintigraphy exam can locate the point of lymphatic obstruction and used to evaluate rate of lymphatic flow.
Treatment
Treatment is multi-focal dependant upon symptoms, severity and location of the hemangioma.
Treatment for the tumor consists of medicines in the corticosteroids class such as prednisolone which are given at varying doses. If this approach is not effective, chemotherapy with vincristine (a drug widely used to treat cancers) is started. If this regimen is not effective, other drugs such as interferon, Cytoxan, and Amicar are used.
Another form of treatment would be embolization of the hemangioma. In this procedure, substances/particles are injected into the blood vessels to stop the blood flow. After that surgical removal may be necessary.
If lymphedema is present and there is damage to the lymphatics, a treatment program for lymphedema management should be initiated. The lymphedema treatment program would include: Manual lymphatic drainage; compression wraps or compression bandages (using short stretch bandages), compression garments, compression sleeves.
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Alternative titles; symbols
KASABACH-MERRITT SYNDROME; KMSWith 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.
Brizel and Raccuglia (1965); David et al. (1983); Propp and Scharfman (1966); Rodriguez-Erdmann et al. (1970)
Victor A. McKusick - updated : 1/10/2000
OMIM
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Kasabach-Merritt syndrome
Important
It 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 reportSynonyms
- 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 1968
Danbury, CT 06813-1968
Tel: (203)744-0100
Fax: (203)798-2291
Tel: (800)999-6673
TDD: (203)797-9590
Email: orphan@rarediseases.org
Internet: http://www.rarediseases.orgNIH/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
Internet: http://www.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
Internet: http://www.geneticalliance.orgHemangioma Support System
c/o Cynthia Schumerth
1484 Sand Acres Drive
DePere, WI 54115
Tel: (920)336-9399
..................................
Department of Dermatology, Radboud University Medical Centre, Nijmegen, The Netherlands.
Kaposiform hemangioendothelioma is a rare vascular tumor in children. Especially, in association with the Kasabach-MerrittPhenomenon it can be life threatening. The management of these patients is very difficult and an aggressive treatment regime is required. Several multimodality and chemotherapeutic regimens have been described but with variable success and many side effects. We present a 6-week-old boy with Kaposiform hemangioendothelioma and Kasabach-Merritt Phenomenon. Ongoing propranolol treatment with only 4 initial courses of vincristine resulted in a remission that lasted at least 1 year.
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Kasabach-Merritt syndrome: therapeutic considerations.PMID: 3108848 [PubMed - indexed for MEDLINE]
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Kasabach-Merrit
Syndrome: A Case Review
Diane M. Szlachetka, RNC, MSN, NNP
ABSTRACT
Hemangiomas are common newborn vascular tumors occurring in up to 2.5 percent of newborns. Most are benign and 70 to 80 percent regress by age seven. Some hemangiomas are life threatening--1 in 300 is associated with coagulopathy. Kasabach-Merritt syndrome (KMS), associated with hemangioma, disseminated intravascular coagulopathy, microgangiopathic anemia, and thrombocytopenia, can have profound sequelae. Morbidity and mortality are influenced by the anatomic location and size of the hemangioma. Untreated KMS has a 10-37 percent mortality rate. Bleeding secondary to the consumptive coagulopathy is the primary cause of death in these patients. As of 1997, there have been approximately 205 cases of KMS reported in the literature. This case study discusses the pathophysiology, clinical manifestations, diagnostics, treatment modalities, differential diagnosis, and psychosocial considerations of KMS. Neonatal Network (link no longer avaliable)
February 1998
Vol. 17, No. 1
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External Links
................
Kasabach-Merritt Syndrome
http://emedicine.medscape.com/article/202455-overview
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Kasabach-Merritt Syndrome
................
................
http://www.springerlink.com/content/jt01121123351336/
................
http://www.ncbi.nlm.nih.gov/pubmed/21697235
................
http://www.jstage.jst.go.jp/article/nmc/51/5/51_375/_article
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http://www.annals.edu.sg/pdf/39VolNo12Dec2010/V39N12p941.pdf
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Classification and codes:
IC9-9
OMIM http://omim.org/entry/141000
==============
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