Kasabach-Merritt Syndrome

Rapidly growing vascular lesion along the midline axis of the neck, upper trunk, and extremities that is characterized by CONSUMPTION COAGULOPATHY; THROMBOCYTOPENIA; and HEMOLYTIC ANEMIA. It is often associated with infantile Kaposiform HEMANGIOENDOTHELIOMA and other vascular tumors such as tufted ANGIOMA.

Also Known As:

Hemangioma-Thrombocytopenia Syndrome; Hemangioma Thrombocytopenia Syndromes; Kasabach Merritt Phenomenon; Kasabach Merritt Syndrome; Phenomenon, Kasabach-Merritt; Syndrome, Hemangioma Thrombocytopenia; Syndrome, Kasabach-Merritt; Syndrome, Thrombocytopenia-Hemangioma; Syndromes, Hemangioma Thrombocytopenia; Syndromes, Thrombocytopenia-Hemangioma; Thrombocytopenia Hemangioma Syndrome; Thrombocytopenia-Hemangioma Syndromes; Hemangioma Thrombocytopenia Syndrome; Kasabach-Merritt Phenomenon; Thrombocytopenia-Hemangioma Syndrome

Networked: 255 relevant articles (22 outcomes, 7 trials/studies)

Relationship Network

Disease Context: Research Results

Related Diseases

1.	Hemangioma (Angioma)
2.	Neoplasms (Cancer)
3.	Kaposiform Hemangioendothelioma
4.	Hemorrhage
5.	Disseminated Intravascular Coagulation

Experts

1.	Li, Kai: 5 articles (07/2022 - 08/2014)
2.	Dong, Kuiran: 4 articles (07/2020 - 08/2014)
3.	Wang, Zuopeng: 4 articles (07/2020 - 08/2014)
4.	Zheng, Shan: 4 articles (07/2020 - 08/2014)
5.	Cui, Jie: 4 articles (06/2020 - 01/2010)
6.	Boccara, O: 3 articles (01/2022 - 04/2016)
7.	Hadj-Rabia, S: 3 articles (01/2022 - 04/2016)
8.	Zhang, Jing: 3 articles (05/2018 - 03/2013)
9.	Gao, Y: 3 articles (01/2018 - 11/2004)
10.	Xiao, Xianmin: 3 articles (04/2015 - 08/2014)

Drugs and Biologics

Drugs and Important Biological Agents (IBA) related to Kasabach-Merritt Syndrome:

1.	Sirolimus (Rapamycin)FDA Link 06/01/2020 - "Recently, promising results have shown that sirolimus had been successfully used to treat Kasabach-Merritt phenomenon without significant toxicity. " 12/01/2023 - "This retrospective study aimed to observe the efficacy of transcatheter arterial chemoembolization (TACE) combined with sirolimus in the treatment of haemangioma combined with the Kasabach-Merritt phenomenon (KMP). " 12/01/2023 - "Efficacy of transcatheter arterial chemoembolization combined with sirolimus for treating Kasabach-Merritt phenomenon in infants, a retrospective study." 09/01/2018 - "Efficacy of sirolimus in an infant with Kasabach-Merritt phenomenon." 01/01/2022 - "Management of sirolimus treatment for tumours associated with Kasabach-Merritt phenomenon."
2.	Vincristine (Oncovin)FDA LinkGeneric 10/01/2014 - "Successful management of a small infant with Kasabach-Merritt phenomenon using vincristine: a case report." 05/01/2012 - "Outcome of Kasabach-Merritt phenomenon: the role of vincristine as monotherapy: report of a case." 08/01/2010 - "Out of the 7 cases treated with vincristine, 3 patients had associated Kasabach-Merritt phenomenon (43%). " 05/23/2008 - "Six weeks treatment with vincristine in a dose of 0.5 mg/kg/week followed by surgical excision may be the best management in selected cases of Kasabach-Merritt syndrome." 05/23/2008 - "Successful treatment of kasabach-merritt syndrome with vincristine and surgery: a case report and review of literature."
3.	Adrenal Cortex Hormones (Corticosteroids)IBA 10/01/2012 - "Systemic corticosteroid and surgery may be considered an option for Kasabach-Merritt syndrome, although well-designed studies are needed to quantify the benefits and risks of this treatment." 03/01/2013 - "Successful treatment of Kasabach-Merritt syndrome with transarterial embolization and corticosteroids." 07/10/2020 - "Corticosteroid is indicated in the episode of acute Kasabach-Merritt syndrome after the failure of massive blood transfusion." 03/01/2013 - "The combination of transarterial embolization and corticosteroid therapy should be considered as an option for Kasabach-Merritt syndrome." 12/01/2012 - "Successful treatment of Kasabach-Merritt phenomenon with intralesional corticosteroid injections: a case series."
4.	Prednisone (Sone)FDA LinkGeneric 05/01/1986 - "Is prednisone the treatment of first choice in Kasabach-Merritt syndrome in neonates?" 10/01/1991 - "These observations have important implications for the management of patients with Kasabach-Merritt syndrome because they show that even in severe cases blood transfusions can be avoided by the use of prednisone and EACA." 07/01/1985 - "A giant haemangioma of the thigh in a 9-month-old infant girl with the Kasabach-Merritt syndrome was operated upon with temporary clamping of the common iliac artery after previous attempts at treatment with external compression, prednisone and radiotherapy had failed. " 10/01/1991 - "Successful treatment of Kasabach-Merritt syndrome with prednisone and epsilon-aminocaproic acid." 09/02/2017 - "Objective: To analyze the clinical value of sirolimus plus prednisone for the treatment of the refractory kaposiform hemangioendothelioma(RKHE) and Kasabach-Merritt syndrome(KMS). "
5.	Ticlopidine (Ticlid)FDA LinkGeneric 10/01/2012 - "Kaposiform hemangioendothelioma with Kasabach-Merritt phenomenon: successful treatment with vincristine and ticlopidine." 05/01/2009 - "Successful management of Kaposiform hemangioendothelioma with Kasabach-Merritt phenomenon using vincristine and ticlopidine." 05/01/2009 - "We present a case of a newborn with a rapid tumor growth and a life-threatening Kasabach-Merritt syndrome with a progressive remission after treatment with vincristine and ticlopidine." 09/01/2013 - "Long-term outcome of vincristine-aspirin-ticlopidine (VAT) therapy for vascular tumors associated with Kasabach-Merritt phenomenon." 11/01/2010 - "Vincristine-ticlopidine-aspirin: an effective therapy in children with Kasabach-Merritt phenomenon associated with vascular tumors."
6.	Aspirin (Acetylsalicylic Acid)FDA LinkGeneric 01/01/2013 - "We present two patients with TA without Kasabach-Merritt phenomenon (KMP) treated with low dose aspirin, which resulted in improved appearance, decreased bulk, and resolution of symptoms. " 07/04/1992 - "Aspirin-ticlopidin in Kasabach-Merritt syndrome." 09/01/2013 - "Long-term outcome of vincristine-aspirin-ticlopidine (VAT) therapy for vascular tumors associated with Kasabach-Merritt phenomenon." 11/01/2010 - "Vincristine-ticlopidine-aspirin: an effective therapy in children with Kasabach-Merritt phenomenon associated with vascular tumors." 09/01/2013 - "This study aimed to clarify the combinatorial treatment effect of agents as aspirin and ticlopidine associated with vincristine in the management of Kasabach-Merritt phenomenon (KMP), a severe thrombocytopenic coagulopathy that occurs in the presence of an enlarging vascular tumor such as kaposiform hemangioendothelioma (KHE) and tufted angioma (TA). "
7.	Thalidomide (Thalomid)FDA Link 04/01/2019 - "Prednisone combined with vincristine and thalidomide may be an effective treatment for Kasabach-Merritt syndrome, but further studies are needed to verify the use of this regimen." 04/01/2019 - "Successful treatment of an adult with Kasabach-Merritt syndrome using thalidomide, vincristine, and prednisone." 04/01/2019 - "A woman with Kasabach-Merritt syndrome presented with a recurrent subcutaneous mass and disseminated intravascular coagulation, and was treated with prednisone, vincristine and thalidomide. "
8.	Propranolol (Inderal)FDA LinkGeneric 10/27/2017 - "Orally administered propranolol as monotherapy or in combination with other therapeutic modalities may play a key role in the treatment of Kasabach-Merritt syndrome." 10/27/2017 - "We present a case of Kasabach-Merritt syndrome treatment with orally administered propranolol. " 10/27/2017 - "Propranolol in the preoperative treatment of Kasabach-Merritt syndrome: a case report." 01/01/2014 - "Successful treatment of mild pediatric kasabach-merritt phenomenon with propranolol monotherapy." 01/01/2021 - "We present the case of a newborn with a hepatic hemangioma and Kasabach-Merritt syndrome with an excellent response and tolerance to propranolol. "
9.	InterferonsIBA 05/01/2015 - "[Is alfa-interferon still current in the management of Kasabach-Merritt syndrome?]." 07/01/2010 - "Kasabach merritt syndrome: management with interferon." 05/01/1997 - "Successful interferon therapy in a neonate with life-threatening Kasabach-Merritt syndrome." 09/01/1995 - "Interferon therapy in an infant with Kasabach-Merritt syndrome." 12/01/1992 - "Interferon alpha-2a therapy of consumptive coagulopathy in Kasabach-Merritt syndrome."
10.	Prednisolone (Predate)FDA LinkGeneric 06/01/2013 - "The patient was diagnosed with Kasabach-Merritt syndrome, and prednisolone was commenced as a first-line treatment. " 12/01/2017 - "Clinically, Kasabach-Merritt syndrome (KMS) was suspected, and immediately he was treated with daily prednisolone (PSL) 1 mg/kg and recombinant thrombomodulin without response. " 11/01/2010 - "He was diagnosed with Kasabach-Merritt syndrome and treated with intravenous dexamethasone at 0.32 mg/kg/day (equivalent to prednisolone 2.0 mg/kg/day). " 12/01/2017 - "Successful combination therapy of propranolol and prednisolone for a case with congenital Kasabach-Merritt syndrome." 09/01/2003 - "Treatment of Kasabach-Merritt syndrome: a stepwise regimen of prednisolone, dipyridamole, and interferon."

Therapies and Procedures

1.	Therapeutics 08/01/1993 - "Improvement in manifestations of this Kasabach-Merritt syndrome is difficult to assign to one of the several therapies. " 08/26/2022 - "Combination therapy for pediatric patients with Kasabach-Merritt phenomenon: A single-center retrospective study." 10/01/2013 - "Kasabach-Merritt phenomenon (KMP) is a rare condition and optimal treatments have not yet been established, especially for cases that are unresponsive to first-line therapy. " 09/27/2011 - "[Combined and sequential therapy for Kasabach-Merritt syndrome]." 07/14/2009 - "To summarize the efficacy and safety of employing pneumatic compression therapy in infants with Kasabach-Merritt phenomenon (KMP). "
2.	Liver Transplantation 04/04/2014 - "However, liver transplantation as a potential cure for a stable patient without Kasabach-Merritt syndrome remains debatable. " 01/01/1997 - "We describe a case of giant cavernous haemangioma of the liver with disseminated intravascular coagulopathy (Kasabach-Merritt syndrome) which was cured by orthotopic liver transplant. " 05/01/2011 - "In selected patients, especially in case of Kasabach-Merritt syndrome, there is a good indication for liver transplantation." 01/01/1997 - "Treatment of a giant haemangioma of the liver with Kasabach-Merritt syndrome by orthotopic liver transplant a case report." 01/01/1997 - "Orthotopic liver transplant can be considered for giant hepatic haemangioma with Kasabach-Merritt syndrome when resection is necessary and a partial hepatectomy is not technically feasible."
3.	Radiotherapy 09/01/1997 - "In this study, we retrospectively investigated the treatment outcome of radiation therapy for seven neonates with Kasabach-Merritt syndrome. " 03/01/2022 - "Reports on results of radiation therapy (RT) for Kasabach-Merritt syndrome (KMS) are limited. " 03/01/2022 - "Long-term outcomes of low-dose radiotherapy in Kasabach-Merritt syndrome." 10/01/2016 - "Successful low-dose radiotherapy treatment for Kasabach-Merritt syndrome." 02/01/2009 - "Radiotherapy remains an important treatment method in Kasabach-Merritt syndrome when patients fail to respond to other treatments."
4.	Platelet Transfusion (Blood Platelet Transfusions) 10/01/2015 - "[Kasabach-Merritt phenomenon (KMP) exacerbated by platelet transfusions]." 04/01/1993 - "Kasabach-Merritt syndrome exacerbated by platelet transfusion." 11/01/2021 - "Paradoxical worsening of symptoms with platelet transfusions and radiological evidences led to the diagnosis of a very rare congenital multifocal vascular tumor, kaposiform hemangioendothelioma (KHE) with Kasabach-Merritt phenomenon (KMP). " 05/01/1997 - "The patient, who was dependent on platelet transfusions, improved quickly after interferon alpha-2a was given at the dosage of 3,000,000 U/m2, with resolution of the Kasabach-Merritt syndrome after three weeks and a 75% decrease of the tumor volume within three months of treatment."
5.	Therapeutic Embolization 01/01/1986 - "Kasabach-Merritt syndrome treated by therapeutic embolization with polyvinyl alcohol." 01/01/1998 - "Absolute ethanol embolotherapy for hemangioma with Kasabach-Merritt syndrome." 01/01/1998 - "This case illustrates that absolute ethanol embolotherapy is an effective treatment for hemangioma with Kasabach-Merritt syndrome and may be life-saving for those with lesions refractory to medical therapy."