Kasabach-Merritt Phenomenon

Yasui, Naoko; Koh, Katsuyoshi; Kato, Motohiro; Park, Myoung-ja; Tomizawa, Daisuke; Oshima, Koichi; Uchisaka, Naoki; Gocho, Yoshihiro; Arakawa, Ayumu; Seki, Masafumi; Oguma, Eiji; Kishimoto, Hiroshi; Watanabe, Shôji; Kikuchi, Akira; Hanada, Ryoji

doi:10.1097/mph.0b013e318281558e

Cited by 27 publications

(7 citation statements)

References 8 publications

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“…Less than 15 cases of exclusively mediastinal KHE have been reported, in addition to a number of cases where large KHEs extended contiguously from the neck into the mediastinum [ 51 , 77 , 81 , 89 , 114 , 140 , 210 , 211 , 220 , 226 , 234 ]. The reported mediastinal KHE cases were exclusively seen in infants (0–16 months of age) with almost equal sex distribution.…”

Section: Vascular Tumoursmentioning

confidence: 99%

Mesenchymal tumours of the mediastinum—part II

et al. 2015

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Section: Vascular Tumoursmentioning

confidence: 99%

Mesenchymal tumours of the mediastinum—part II

et al. 2015

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“…Fatal complications of the syndrome are diverse, including hemorrhage, cardiac failure, and invasion of vital organs. Although different therapies for KMS have shown variable responses and outcomes [101112131415161718], the optimal treatment and consensus guidelines for KMS have not been established, a circumstance largely attributable to the rarity of the syndrome. Currently, various treatment options available for KMS include medical treatments, vascular embolization, radiation therapy, and surgery [56789].…”

Section: Discussionmentioning

confidence: 99%

Excellent outcome of medical treatment for Kasabach-Merritt syndrome: a single-center experience

Kim

Choi

et al. 2016

Blood Res

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BackgroundKasabach-Merritt syndrome (KMS) is a rare but life-threatening illness. The purpose of this study is to report our single-center experience with KMS.MethodsWe reviewed the medical records of 13 patients who were diagnosed with KMS between 1997 and 2012 at Samsung Medical Center. Treatment response was defined as follows: 1) hematologic complete response (HCR) – platelet count >130×109/L without transfusion; 2) clinical complete response (CCR) – complete tumor disappearance or small residual vascular tumor displaying lack of proliferation for at least 6 months after treatment discontinuation.ResultsParticipants included 7 male and 6 female patients. The median initial hemoglobin levels and platelet counts were 9.7 g/dL (range, 6.6–11.6 g/dL) and 11×109/L (range, 3–38×109/L), respectively. Twelve patients received corticosteroid and interferon-alpha as initial treatment, and the remaining patient received propranolol instead of corticosteroid. Two patients with unsatisfactory response to the initial treatment received weekly vincristine. Successful discontinuation of medication was possible at a median of 301 days (range, 137–579) in all patients except one who was lost to follow-up. The median times to achieve HCR and CCR were 157 days and 332 days, respectively. The probabilities of achieving HCR and CCR were 77% and 54% at 1 year, and 88% and 86% at 2.5 years, respectively.ConclusionThe prognosis of KMS in our cohort was excellent. Our data suggest that individualized treatment adaptation according to response may be very important for the successful treatment of patients with KMS.

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“…Vincristine is usually used in combination with other therapies for severe, life-threatening cases because of its good tolerance and response. Several studies used vincristine monotherapy for KMP with good response rates of 86–100% [8, 11], but this agent has not been used widely because of its neurotoxicity and other side effects [8, 12, 14]. …”

Section: Discussionmentioning

confidence: 99%

Successful Treatment of Mild Pediatric Kasabach-Merritt Phenomenon with Propranolol Monotherapy

Choeyprasert

Natesirinilkul

Charoenkwan

2014

Case Reports in Hematology

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Kasabach-Merritt phenomenon (KMP) is relatively rare in childhood and adolescents with high mortality rate because of its hemorrhagic complications and unresponsiveness to treatments such as corticosteroids, vincristine, intravascular embolization, and/or surgery. Propranolol, a β-adrenergic receptor blocker, has a promising efficacy against vascular tumors such as infantile hemangiomas. But limited and variable data has been reported regarding the role of propranolol in treatment of KMP. We herein reported the successful treatment of mild pediatric KMP with propranolol monotherapy in a case of a five-week-old child with kaposiform hemangioendothelioma with successful treatment of both clinical and hematologic responses. After eight months of follow-up, patient still had stable cutaneous lesion while receiving propranolol monotherapy. Regular hematologic monitoring was done in order to detect any late relapse of the disease. Six months after discontinuation of propranolol, patient has still remained free of hematologic relapse, and primary cutaneous lesion has become a pale pink, 1 cm sized skin lesion.

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Kasabach-Merritt Phenomenon

Abstract: Chemotherapy seems to be the most effective therapy for steroid-resistant KMP cases.

Cited by 27 publications

References 8 publications

Mesenchymal tumours of the mediastinum—part II

Mesenchymal tumours of the mediastinum—part II

Excellent outcome of medical treatment for Kasabach-Merritt syndrome: a single-center experience

Successful Treatment of Mild Pediatric Kasabach-Merritt Phenomenon with Propranolol Monotherapy

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