Haemangioma with thrombocytopenia (Kasabach-Merritt syndrome).

David, T J; Evans, D. I. K.; Stevens, Robert G.

doi:10.1136/adc.58.12.1022

Cited by 17 publications

(2 citation statements)

References 4 publications

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“…It is recommended as emergency treatment to alleviate hazardous coagulopathies and life‐ or function‐threatening hemangiomas 2,8,18,19. The failure of other therapies particularly steroids is a further indication 9,20.…”

Section: Discussionmentioning

confidence: 99%

Radiotherapy for life‐threatening mediastinal hemangioma with Kasabach–Merritt syndrome

Kwok‐Williams¹,

Perez

Squire

et al. 2006

Pediatric Blood & Cancer

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Kasabach-Merritt Syndrome (KMS) originally referred to a hemangioma associated with life-threatening thrombocytopenia and a consumption coagulopathy. Treatment includes surgery, steroids, and alpha interferon. However response may be unsatisfactory and mortality rates of 10-37% have been reported. We report a case of a 4-month-old male whose disease had not responded to steroids and alpha interferon. He required ventilation, and his condition was deteriorating. It was thought that he would probably die of his condition. He was treated with 9 Gy in three fractions weekly of radiotherapy (RT). Clinical response was good and he is well and free from progression 4 years and 7 months after RT. A review of the literature on the role of RT in the management of hemangiomas is presented.

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Section: Discussionmentioning

confidence: 99%

Radiotherapy for life‐threatening mediastinal hemangioma with Kasabach–Merritt syndrome

Kwok‐Williams¹,

Perez

Squire

et al. 2006

Pediatric Blood & Cancer

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“…It has been concluded that in patients not responding to corticosteroids, alternative therapies (e.g. radiotherapy and/or interferon alpha) should be used within 3-4 weeks [30].…”

Section: Steroidsmentioning

confidence: 99%

Kasabach–Merritt syndrome: a review of the therapeutic options and a case report of successful treatment with radiotherapy and interferon alpha

Hesselmann¹,

Micke²,

Marquardt³

et al. 2002

BJR

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We describe the successful treatment of a neonate with Kasabach-Merritt syndrome who received local irradiation and interferon alpha therapy after failure of corticosteroid treatment. A male neonate, born after an uneventful pregnancy, had a huge haemangioma involving the upper right cervical region as well as severe thrombocytopenia. He was treated with corticosteroids, interferon alpha and radiotherapy. Prednisolone therapy (5 mg kg(-1) day(-1)) was started at 41 days of age. No therapeutic effect was observed after 2 weeks. At this time the tumour size had increased dramatically, platelet counts had decreased progressively and coagulation abnormalities had developed. Because corticosteroid therapy had been ineffective and the child was in a life-threatening condition, irradiation was delivered up to a total dose of 9.5 Gy in five fractions. Simultaneously, prednisolone therapy was slowly decreased and interferon alpha therapy (3 million U m(-2) day(-1)) was started and continued for 6 weeks. After irradiation with 9.5 Gy and beginning interferon alpha therapy, the tumour decreased in size and coagulation parameters normalized within 4 weeks. 6 months later, platelet counts and coagulation parameters were still normal. The tumour had further decreased in size. No acute severe side effects were observed. Radiation therapy combined with interferon alpha treatment is an alternative treatment modality when high dose corticoid steroid therapy has been ineffective in patients with Kasabach-Merritt syndrome, despite the risks of growth delay and secondary malignancy. In children showing no response to corticosteroids, radiotherapy and/or interferon alpha should be considered in Kasabach-Merritt syndrome.

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Therapeutic Efficacy of Interferon Alfa-2b in Infants With Life-Threatening Giant Hemangiomas

Tamayo¹

1997

Arch Dermatol

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Haemangioma with thrombocytopenia (Kasabach-Merritt syndrome).

Abstract: that in the long term and on the basis of the radiographic evidence our patient's bone structure may be normal.

Cited by 17 publications

References 4 publications

Radiotherapy for life‐threatening mediastinal hemangioma with Kasabach–Merritt syndrome

Radiotherapy for life‐threatening mediastinal hemangioma with Kasabach–Merritt syndrome

Kasabach–Merritt syndrome: a review of the therapeutic options and a case report of successful treatment with radiotherapy and interferon alpha

Therapeutic Efficacy of Interferon Alfa-2b in Infants With Life-Threatening Giant Hemangiomas

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