Hemangioma thrombocytopenia syndrome
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 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.
Alternative titles; symbolsKASABACH-MERRITT SYNDROME; KMS
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.
Victor A. McKusick - updated : 1/10/2000
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
- Kasabach-Merritt Syndrome
- Thrombocytopenia-Hemangioma Syndrome
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.
National Organization for Rare Disorders, Inc.
55 Kenosia Ave
PO Box 1968
Danbury, CT 06813-1968
NIH/National Heart, Lung and Blood Institute
31 Center Drive MSC 2480
Building 31A Rm 4A16
Bethesda, MD 20892-2480
4301 Connecticut Avenue NW
Washington, DC 20008-2304
...................................Kasabach-Merritt syndrome: therapeutic considerations.
PMID: 3108848 [PubMed - indexed for MEDLINE]
Syndrome: A Case Review
Diane M. Szlachetka, RNC, MSN, NNP
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)
Vol. 17, No. 1
Classification and codes:
Join us as we work for lymphedema patients everywhere:
Advocates for Lymphedema
Dedicated to be an advocacy group for lymphedema patients. Working towards education, legal reform, changing insurance practices, promoting research, reaching for a cure.
Lymphedema People / Advocates for Lymphedema
For information about Lymphedema
For Information about Lymphedema Complications
For Lymphedema Personal Stories
For information about How to Treat a Lymphedema Wound
For information about Lymphedema Treatment
For information about Exercises for Lymphedema
For information on Infections Associated with Lymphedema
For information on Lymphedema in Children
Lymphedema People - Support Groups
The time has come for families, parents, caregivers to have a support group of their own. Support group for parents, families and caregivers of chilren with lymphedema. Sharing information on coping, diagnosis, treatment and prognosis. Sponsored by Lymphedema People.
No matter how you spell it, this is another very little understood and totally frustrating conditions out there. This will be a support group for those suffering with lipedema/lipodema. A place for information, sharing experiences, exploring treatment options and coping.
Come join, be a part of the family!
MEN WITH LYMPHEDEMA
If you are a
man with lymphedema; a man with a loved one with lymphedema who you are
to help and understand come join us and discover what it is to be the
instead of the sufferer of lymphedema.
Support group for parents, patients, children who suffer from all forms of lymphangiectasia. This condition is caused by dilation of the lymphatics. It can affect the intestinal tract, lungs and other critical body areas.
Disorders Support Group @ Yahoo Groups
While we have a number of support groups for lymphedema... there is nothing out there for other lymphatic disorders. Because we have one of the most comprehensive information sites on all lymphatic disorders, I thought perhaps, it is time that one be offered.
Information and support for rare and unusual disorders affecting the lymph system. Includes lymphangiomas, lymphatic malformations, telangiectasia, hennekam's syndrome, distichiasis, Figueroa
syndrome, ptosis syndrome, plus many more. Extensive database of information available through sister site Lymphedema People.
Lymphedema People New Wiki Pages
Have you seen
our new “Wiki”
yet? Listed below
are just a sample
of the more than 140 pages now listed in our Wiki section. We are also
on hundred more. Come
and take a
not for Lymphedema
People Online Support Groups
Lymphatic Drainage (MLD) and Complex Decongestive Therapy (CDT)
Associated with Lymphedema
How to Treat a
Infections Associated with Lymphe dema
para-aortic lymph node dissection (EPLND)
Needle Biopsy - Fine Needle Aspiration
Lymphedema Gene VEGFC
Lymphedema Gene SOX18
Home page: Lymphedema People
Page Updated: Dec. 23, 2011