Treatment Of Kasabach-Merritt Phenomenon Research Articles

Cocktail therapy with prednisolone, vincristine and sirolimus for Kasabach‑Merritt phenomenon in 10 infants.

Kasabach-Merritt phenomenon (KMP) is a life-threatening condition caused by rare vascular tumors. To reduce drug resistance observed in monotherapy of KMP with prednisone, vincristine (VCR) or sirolimus, the present study evaluated the efficacy and safety of triad therapy in the treatment of KMP. A total of 10 KMP infants managed with prednisolone, VCR and sirolimus in The Second Affiliated Hospital of Xi'an Jiaotong University (Xi'an, China) between April 2017 and August 2021 were retrospectively reviewed. The three female and seven male infants with KMP underwent cocktail therapy with prednisone, VCR and sirolimus. At diagnosis, the infants, aged 49.1±41.0 days, showed laboratory test results with platelet counts 22±15.4x109/l, fibrinogen 81.7±26.9 mg/dl and D-dimer 38649±13443.6 ng/ml. The average maximal diameter of the tumors at diagnosis was 84.5±25.1 mm. KMP risk is increased by large tumors with deep lesions infiltrating the muscle. Platelet counts normalized after a median 10 days (range, 5-69 days) of treatment. With combination therapy maintained for 46.8±24.4 days, ultrasound showed that the thickness of the tumors decreased by 51% from 28.9±12.1 to 13.9±6.2 mm. Neutropenia and gastrointestinal disorders were the most common adverse effects. The present study found that the cocktail therapy with prednisolone, VCR and sirolimus has favorable tolerance and efficacy for life-threatening KMP. Once a stable condition has been achieved, cocktail therapy should be replaced by sirolimus monotherapy to reduce potential side effects.

Recent advances in the diagnosis and treatment of Kasabach-Merritt phenomenon

Kasabach - Merritt phenomenon(KMP), also known as Kasabach - Merritt syndrome, is a rare syndrome associated with Kaposiform haemangioendothelioma or tufted angioma, and characterized by thrombocytopenia and consumptive coagulopathy.KMP onsets early and progresses quickly.If diagnosis and treatment delayed, it can be life-threatening.In this paper, the progress of clinical manifestations, pathogenesis, diagnosis and treatment of KMP are summarized. Key words: Kasabach-Merritt phenomenon; Kaposiform hemangioendothelioma; Tufted angioma; Child

Successful Treatment of Kasabach-Merritt Phenomenon Arising From Kaposiform Hemangioendothelioma by Sirolimus

Successful Treatment of Kasabach-Merritt Phenomenon Arising From Kaposiform Hemangioendothelioma by Sirolimus

Treating kaposiform hemangioendothelioma with Kasabach-Merritt phenomenon by intralesional injection of absolute ethanol.

Kasabach-Merritt phenomenon (KMP) is characterized by thrombocytopenia, microangiopathic hemolytic anemia, consumptive coagulopathy, and an enlarging vascular lesion. It is a rare and life-threatening disease of vascular tumor. The purpose of this study was to assess the effectiveness of absolute ethanol in the treatment of KMP. We treated 8 pediatric patients with KMP by using absolute ethanol injections, whose cases failed previously when applied steroid therapy. We reviewed the clinical and laboratory data of these 8 cases at Nanjing Children's Hospital in China. Eight pediatric patients (5 female and 3 male) showed tumor regression after treatments. Core needle biopsy had been performed on the 8 patients, and the results included 7 kaposiform hemangioendotheliomas and one tufted angioma. All patients had an increase in platelet count. All coagulopathies were corrected. Complications included one patient with flush on face, one patient with needle scars, and one patient with a small area of local tissue necrosis. Absolute ethanol therapy presents a safe option in the treatment of KMP. Treatment-associated complications seemed to be reversible and acceptable by severity levels. Direct intralesional injection of absolute ethanol provides a simple and reliable alternative treatment for KMP among infants and may be used as the second-line therapy.

Successful treatment of mild pediatric kasabach-merritt phenomenon with propranolol monotherapy.

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.

The therapic effect of the combination of endovascular embolization and hormone in Kasabach-Merritt phenomenon

Objective To explore the therapic effect of the combination of endovascular embolization and clinical hormone in treatment of Kasabach-Merritt phenomenon (KMP),and analyze the advantages. Methods Six cases with KMP from May 2010 to June 2012 were retrospectively analyzed.All the patients underwent large dose hormone shock therapy after admission for 7—10 d.Subsequently,selective endovascular embolization was performed using Seldinger technique under general anesthesia.Then,the hormone therapy was continued for 2 weeks after embolization.The platelet count and the effect were recorded.If the area of the tumor reduction is less than 50%,3—4 courses of local hardening treatment was conducted for the residual tumor.The local treatment used multipoint puncturing of the tumor and injection drugs under X-ray fluoroscopic monitoring until the tension of local vascular increased. Results On DSA,the lesions of all the 6 cases showed rich blood supply with a large number of hybrid distribution of tumor blood vessels.The lesions disappeared in 4 cases after 1—2 weeks combination therapy and no recurrence for 0.5—1.0 year follow-up.Two cases whose tumor reduced less than 50% after combination therapy received local hardening treatment,and faded after 3—4 courses.The number of platelet for all patients kept normal and the spirit of the patients showed great improvement,the bleeding tendency and local soft tissue swelling was in remission.Subcutaneous blood stasis and petechiae disappeared.There were no serious adverse reaction and complications. Conclusion Combination therapy with endovascular embolization and clinical hormone for Kasabach-Merritt phenomenon has a good curative effect. Key words: Hemangioendothelioma; Embolization,therapeutic; Hormones

Kasabach-merritt phenomenon: a retrospective study of treatment with vincristine.

Kasabach-Merritt phenomenon (KMP) is characterized by profound thrombocytopenia, microangiopathic hemolytic anemia, a consumptive coagulopathy, and an enlarging vascular lesion. The syndrome develops in infancy and is associated with a high morbidity and mortality rate. The purpose of this study was to assess the effectiveness of vincristine in the treatment of KMP. We retrospectively reviewed the clinical and laboratory data of 15 patients with KMP treated with vincristine at 9 institutions across the United States, South America, and Europe. All 15 patients had profound thrombocytopenia and consumption of fibrinogen at presentation. Ten patients had biopsies of their lesions, and results included five (33.3%) kaposiform hemangioendotheliomas, three (20%) tufted angiomas, one lesion (6.7%) with features of both kaposiform hemangioendothelioma and tufted angioma, and one (6.7%) unclassified vascular tumor. All 15 patients had an increase in platelet count of at least 20,000 with an average response time of 4.0 weeks after initiation of vincristine therapy. Thirteen patients had an increase in fibrinogen level of 50 mg/dL with an average response time of 3.4 weeks. In 13 patients there was a significant decrease in the size of the vascular lesion. The average duration of treatment was 21.5 (+/-12.6) weeks. Four patients (26%) relapsed. All four were successfully treated with a second course of vincristine. Complications included one patient with abdominal pain, one patient with transient loss of deep tendon reflexes, and one patient with irritability. Vincristine presents a safe and sometimes effective treatment option in the management of KMP.