Use of radiotherapy to treat life‐threatening Kasabach–Merritt syndrome

Leong, Eugene; Bydder, Sean

doi:10.1111/j.1754-9485.2009.02042.x

Cited by 21 publications

(16 citation statements)

References 21 publications

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“…Radiation therapy was curative in the initial case of KMP (Kasabach & Merritt, ) and has proven effective in many other case reports and small series since (El‐Dessouky et al , ; Leong & Bydder, ). In a series of 28 patients, when used in combination with steroids, there was a 75% response rate, with no radiation‐induced side effects over a median follow‐up of more than 6 years (Shin et al , ).…”

Section: Mechanical Options and Radiotherapymentioning

confidence: 99%

Recent advances in the pathobiology and management of Kasabach–Merritt phenomenon

et al. 2015

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SummaryKasabach-Merritt Phenomenon (KMP) refers to the clinical constellation of thrombocytopenia, consumptive coagulopathy and purpura associated with Kaposiform haemangioedothelioma or tufted angioma, but not the more common infantile haemangioma. It shows a variable and unpredictable response to traditional pharmacological agents, such as steroids, vincristine or interferon alpha 2a or 2b. More recently, the interaction between platelets and endothelial cells and the proangiogenic phenotype that results has been recognized to underly the pathogenesis of this disorder. Recent efforts have attempted to target the platelet by using antiplatelet agents and by the withholding of platelet transfusions even in those patients who have significant thrombocytopenia and laboratory evidence of coagulopathy. Excellent response rates and prompt results have been achieved by combining antiplatelet therapy with vincristine, without the need for steroid use. This synergistic approach moves away from the conventional wisdom of treating the underlying lesion to control the coagulopathy. Sirolimus, which is directed against the PI3/AKT/ mTOR downstream signalling pathway involved in lymphangiogenesis, has also shown promising results, although further study is needed.

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Section: Mechanical Options and Radiotherapymentioning

confidence: 99%

Recent advances in the pathobiology and management of Kasabach–Merritt phenomenon

et al. 2015

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“…Historically, radiation therapy has been associated with multiple long-term side effects when used in pediatric population [60,100,[113][114][115][116] its use in adults has been more liberal. In a series of 28 patients, concurrent radiation and steroids demonstrated a 75% response rate, with no radiation-induced side effects over a median follow-up of more than 6 years [117]. With new advancements in radiotherapy, there is minimal radiation delivered to uninvolved organs and this can lead to more liberal use for adult population.…”

Section: Radiationmentioning

confidence: 99%

Clinical Review: Management of Adult Kasabach-Merritt Syndrome Associated with Hemangiomas

Master¹,

Kallam²,

Eo³

et al. 2017

J Blood Disord Transfus

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Kasabach-Merritt syndrome (KMS) consists of a clinical trial of capillary hemangioma, thrombocytopenia and disseminated intravascular coagulation. KMS occurs most commonly in the pediatric population, and its occurrence in adults is rare. Specific guidelines or randomized clinical trials guiding clinical management of KMS in adult patients are lacking. This manuscript provides a comprehensive review of KMS and discusses recent advances in the medical management of KMS. We also propose a systematic therapeutic approach which would serve as a guide in the management of adult patients with KMS caused by hemangiomas.

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“…Although some small-cohort studies have reported no detection of cancer in response to radiation, 8,9 a retrospective study of 18 000 patients treated with radiotherapy for hemangioma during childhood noted a significantly increased dose-dependent risk of breast cancer, brain tumors, and bone tumors. 10 Therefore, Mitsuhashi et al 9 recommended that the total irradiation dose be kept as low as possible, not exceeding 10 Gy.…”

Section: Discussionmentioning

confidence: 99%

Successful low‐dose radiotherapy treatment for Kasabach–Merritt syndrome

Yamamoto

Akiyama

Kaneko

et al. 2016

Pediatrics International

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Kasabach-Merritt syndrome (KMS) is characterized by hemangioma associated with life-threatening thrombocytopenia, and is a consumptive coagulopathy. Although treatments available include corticosteroids, α-interferon, vincristine, and surgery, response may be unsatisfactory, and the mortality rate remains at approximately 30%. Although radiotherapy has been used effectively for KMS, it may cause growth retardation and secondary malignancy. We report a case of KMS in which hemangioma of the left thigh was successfully treated with low-dose radiotherapy (6 Gy in six fractions, weekly) after failure of corticosteroid therapy. No significant late effects due to the radiotherapy were noted at 5 year follow up. Thus, low-dose radiotherapy remains an important treatment method for KMS when patients fail to respond to other treatments.

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Use of radiotherapy to treat life‐threatening Kasabach–Merritt syndrome

Cited by 21 publications

References 21 publications

Recent advances in the pathobiology and management of Kasabach–Merritt phenomenon

Recent advances in the pathobiology and management of Kasabach–Merritt phenomenon

Clinical Review: Management of Adult Kasabach-Merritt Syndrome Associated with Hemangiomas

Successful low‐dose radiotherapy treatment for Kasabach–Merritt syndrome

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