Kasabach-Merritt syndrome

Synonym:

Hemangioma thrombocytopenia syndrome

Symptoms

Hemangiomas which is a reddish-brown visible red skin lesion.
Thrombocytopenia, a disorder in which there are not enough platelets (blood cells that help clotting).
Coagulopathy is a result of the thrombocytopenia which can lead to heavy and prolonged bleeding.
Lymphedema may be present as a result of lymphatic obstruction from the hemangioma.
Petechiae, bruising, and frank bleeding

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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141000	Links
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.

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 : 1/10/2000

OMIM

http://omim.org/entry/141000

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Kasabach-Merritt syndrome

Hemangioma Thrombocytopenia 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 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 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.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
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.org

Hemangioma Support System
c/o Cynthia Schumerth
1484 Sand Acres Drive
DePere, WI 54115
Tel: (920)336-9399

..................................

Kaposiform hemangioendothelioma with Kasabach-Merritt syndrome: a new indication for propranolol treatment.

May 2011

Hermans DJ, van Beynum IM, van der Vijver RJ, Kool LJ, de Blaauw I, van der Vleuten CJ.

Source

Department of Dermatology, Radboud University Medical Centre, Nijmegen, The Netherlands.

Abstract

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.

http://journals.lww.com/jpho-online/pages/articleviewer.aspx?year=2011&issue=05000&article=00027&type=abstract

...................................

Kasabach-Merritt syndrome: therapeutic considerations.

Larsen EC, Zinkham WH, Eggleston JC, Zitelli BJ.

During the past 15 years we have managed six children with capillary hemangiomas in association with consumptive coagulopathy--the Kasabach-Merritt syndrome. Their ages when first seen ranged from 2 weeks to 4 years with a mean of 19 months. In three of the patients, the hemangiomas remained small for many months and then suddenly enlarged and hemorrhagic diatheses appeared. The duration of the thrombocytopenia ranged from 5 to 20 months, a time span that in some of the patients was influenced by the type of treatment used. Each patient received a variety of therapies including the following: medications to control the coagulopathy; mechanical, cytolytic, and pharmacologic treatment to eradicate the lesion; and blood product support with platelets and cryoprecipitate for severe bleeding. A prompt elevation of the platelet count occurred in three of the patients after the lesion was biopsied. Subtotal resection of the lesion resulted in an immediate increase of the platelet count in one patient. A transient increase in platelet counts and fibrinogen levels was observed in another patient following several embolizations of a portion of the hemangioma. Other modes of therapy were difficult to evaluate, especially because the same therapy applied at different times in the same patient effected a different type of response. Eventually, all of the patients experienced resolution of their lesions and, with this, concomitant reversal of the coagulopathy.

Publication Types:

Case Reports

PMID: 3108848 [PubMed - indexed for MEDLINE]

...................................

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

eMedicine

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Successful anesthetic management for resection of a giant hepatic hemangioma with Kasabach-Merritt syndrome using FloTrac system - Nov 2011

PubMed

http://www.ncbi.nlm.nih.gov/pubmed/22175175

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Successful treatment of Kasabach-Merritt syndrome arising from kaposiform hemangioendothelioma by systemic corticosteroid therapy and surgery. - Sept 2011

http://www.springerlink.com/content/jt01121123351336/

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Kasabach-Merritt syndrome in a term neonate. - June 2011

http://www.ncbi.nlm.nih.gov/pubmed/21697235

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Transarterial embolization for cervical hemangioma associated with Kasabach-merritt syndrome. - 2011

http://www.jstage.jst.go.jp/article/nmc/51/5/51_375/_article

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Calcified subgaleal hematoma with secondary cranial deformity in a patient withKasabach-Merritt phenomenon. - Jan 2011

http://journals.lww.com/jcraniofacialsurgery/pages/articleviewer.aspx?year=2011&issue=01000&article=00045&type=abstract

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Cutaneous angiosarcoma associated with the Kasabach-Merritt syndrome. - Dec. 2010

http://www.annals.edu.sg/pdf/39VolNo12Dec2010/V39N12p941.pdf

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Kasabach-merritt syndrome arising from tufted angioma successfully treated with systemic corticosteroid. - Nov 2010

http://anndermatol.org/journal/viewJournal.html?Vol=022&Num=04&page=426

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Kasabach merritt syndrome: management with interferon. - Jul 2010

http://www.e-ijd.org/article.asp?issn=0019-5154;year=2010;volume=55;issue=3;spage=281;epage=283;aulast=Acharya

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Classification and codes:

IC9-9

2008 ICD-9-CM Diagnosis 287.39

Other primary thrombocytopenia

287.39 is a specific code that can be used to specify a diagnosis
287.39 contains 17 index entries
View the ICD-9-CM Volume 1 287.* hierarchy

Index entries containing 287.39:

Deficiency, deficient
- thrombopoieten 287.39
Disease, diseased - see also Syndrome
- Werlhof's (see also Purpura, thrombocytopenic) 287.39
Frank's essential thrombocytopenia (see also Purpura, thrombocytopenic) 287.39
Hemorrhage, hemorrhagic (nontraumatic) 459.0
- purpura (primary) (see also Purpura, thrombocytopenic) 287.39
Kasabach-Merritt syndrome (capillary hemangioma associated with thrombocytopenic purpura) 287.39
Purpura 287.2
- hemorrhagic (see also Purpura, thrombocytopenic) 287.39
  - nodular 272.7
  - nonthrombocytopenic 287.0
  - thrombocytopenic 287.39
- Werlhof's (see also Purpura, thrombocytopenic) 287.39
Syndrome - see also Disease
- Kasabach-Merritt (capillary hemangioma associated with thrombocytopenic purpura) 287.39
- thrombopenia-hemangioma 287.39
- Werlhof-Wichmann (see also Purpura, thrombocytopenic) 287.39
Thrombocytopenia, thrombocytopenic 287.5
- with
  - giant hemangioma 287.39
- cyclic 287.39
- Kasabach-Merritt 287.39
- sex-linked 287.39
Werlhof's disease (see also Purpura, thrombocytopenic) 287.39
Werlhof-Wichmann syndrome (see also Purpura, thrombocytopenic) 287.39

OMIM http://omim.org/entry/141000

eMedicine med/1221 - ped/1234

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