OBJECTIVETo summarize the characters of Kasabach-Merritt syndrome (KMS) and to evaluate the therapeutic effect of drug therapy combined with surgery.

METHODSFrom 2004 to 2010, 59 cases with KMS, who underwent drug therapy and surgery, were retrospectively studied. The average age of the patients, including 33 male and 26 female (male/female, 1.269/1), was 2.9 months (range, 7 days-2.5 years). 28 cases with maxillofacial lesions were treated with the ligation of external carotid artery and injection of carbonyldiamide and methylprednisolone. 31 cases with lesions at trunks and extremities were treated by excision of lesions. All the patients were followed up for 2.8 years (range, 6.5 months -7.3 years). Therapeutic outcomes were assessed by evaluating platelet counts,size of lesion, function of trunk and limb.

RESULTS58 cases were cured except for one dead case. Emergency operation was given in 4 cases, and selective operation was performed in other cases (55 cases). The thrombocyte count, hemoglobin and blood coagulation function returned to normal within 1-2 weeks. The mental condition, appetite, body weight,sleeping were greatly improved one week after treatment. The size of the lesions decreased gradually after the management of ligation of external carotid artery including 18 cases within 6-12 months and 10 cases within 13-24 months. Long term follow-up studies indicated that there was no recurrent case, and the weight, height, immunity of the patients with good function activities were in keeping with the normal counterparts.

CONCLUSIONSThe drug combined with surgery therapy is a very reliable management with high curative rate, short disease period and minimum side-effect.

Child, Preschool ; Combined Modality Therapy ; Female ; Humans ; Infant ; Infant, Newborn ; Kasabach-Merritt Syndrome ; drug therapy ; surgery ; therapy ; Male ; Retrospective Studies

OBJECTIVE: To study the clinical features, treatment, and prognosis of neonates with Kasabach-Merritt syndrome (KMS), and to provide a reference for optimizing the diagnosis and treatment of this disease. METHODS: A retrospective analysis was performed for the clinical and follow-up data of 16 neonates with KMS who were admitted to the Anhui Children's Hospital, Anhui Medical University, from January 2016 to December 2020. RESULTS: Of the 16 neonates, there were 13 boys (81%) and 3 girls (19%), with an age of 1 hour to 10 days on admission. Among these neonates, 13 (81%) had cutaneous hemangioma (2 in the head and face, 5 in the trunk, and 6 in the extremities) and 3 (19%) had liver hemangioma. The main clinical manifestations of bleeding tendency and scattered petechiae and ecchymosis were observed in 10 neonates (62%). All the 16 neonates had varying degrees of thrombocytopenia and coagulation disorders. They all received glucocorticoid treatment after admission and 7 (44%) of them had response, among whom 4 experienced recurrence. Among the neonates with no response to glucocorticoid treatment, 3 received sirolimus treatment, among whom 1 had the tumor volume reduced by 58.8% after 4 weeks of treatment, with platelet count and coagulation function returning to normal, while 2 had no significant reduction in tumor volume or significant increase in platelet count and achieved a tumor volume reduced by (43.7±0.4)% after 4 weeks of combined treatment with bleomycin arterial embolization, with platelet count and coagulation function returning to normal. After 4 weeks of bleomycin arterial embolization alone for 4 neonates, tumor volume was reduced by (52.0±3.4)%, and platelet count and coagulation function returned to normal. Blunt and sharp dissection was performed for 2 neonates. The tumor was removed completely during surgery in the 2 neonates, with no infection or recurrence after surgery, and platelet count and coagulation function returned to normal. The postoperative pathological examination showed Kaposiform hemangioendothelioma in 1 out of the 2 neonates. CONCLUSIONS KMS has characteristic clinical manifestations, histopathological features, and laboratory examination results. The KMS neonates who are not sensitive to glucocorticoids can achieve a good curative effect through arterial embolization and sirolimus treatment.
Child ; Female ; Hemangioendothelioma ; Humans ; Infant, Newborn ; Kasabach-Merritt Syndrome/therapy* ; Male ; Neoplasm Recurrence, Local ; Retrospective Studies ; Sarcoma, Kaposi

PURPOSE: Primary malignant vascular neoplasms of the spleen are rare. It has been known that the prognosis was very poor and the splenectomy before rupture could increase survival. No effective chemotherapeutic protocol for angiosarcomas has yet to be established but patients with or without metastatic disease may be treated by chemotherapy. MATERIAL AND METHODS: We experienced a case of primary splenic angiosarcoma in a 42-year-old woman with multiple purpuric skin rashes associated with consumptive coagulopathy:the Kasabach-Merritt syndrome. The CT showed spleen is diffusely enlarged and inhomogenously enhanced with multiple metastasis in the liver. The splenectomy was done and angiosarcoma was diagnosed. We treated her with conventional combination chemotherapy and obtained partial response. For additional response, high-dose chemotherapy and stem cell rescue with autologous peripheral blood stem cell transplantation was done. RESULT: Afer splenectomy, platelet count return to normal. The follow up abdominal CT scan after treatment showed complete disappeared multiple metastatic lesions in the both lobe of liver and the patient has continued to do well four months following discharge. CONCLUSION: We herein report our experience of a splenic angiosarcoma whose multiple hepatic metastases responded well to the high-dose chemotherapy.
Adult ; Drug Therapy ; Drug Therapy, Combination ; Exanthema ; Female ; Follow-Up Studies ; Hemangiosarcoma* ; Humans ; Kasabach-Merritt Syndrome* ; Liver ; Neoplasm Metastasis ; Peripheral Blood Stem Cell Transplantation ; Platelet Count ; Prognosis ; Rupture ; Spleen ; Splenectomy ; Stem Cells ; Tomography, X-Ray Computed ; Vascular Neoplasms

OBJECTIVETo summarize the management of infant vascular tumors with Kasabach-Merritt phenomenon (KMP) and to evaluate the effect of drug combined with sclerotherapy.

METHODSFrom Feb. 2007 to Nov. 2014, 25 cases with KMP, who underwent drug therapy combined with sclerotherapy, were retrospectively studied. Oral corticosteroids (2 mg/kg per day) was used as the first-line therapy on all of the patients and intravenous vincristine (1.5 mg/m2 every week) was added when the platelet counts didn't recover obviously after 2-3 weeks. After the recovery of the platelet counts, the patients were admitted for sclerotherapy (average, 4.56 sessions per case) with 100% alcohol (1-3 ml per session), Lauromacrogol (1.25-5 ml per session) and betamethasone (0.25-1 ml per session). All the patients were followed up for 42 months ( range, 9 months to 6.5 years). Therapeutic outcomes were assessed by evaluating platelet counts, size of lesion, function of trunk and limb.

RESULTSAll the 25 cases got obvious recovery in the platelet counts [average, (94.3 ± 18.5) x 10(9)/L] after drug therapy, of which 16 were treated by single oral corticosteroids for 4-7 weeks and 9 were treated by corticosteroids plus intravenous vincristine for 2-5 weeks. Meantime, 11 cases received platelet transfusions, of which 3 were coupled with gamma globulin intramuscularly. During the first admission, each of the 25 cases received 1-4 sessions of sclerotherapy (average, 2.6 sessions each case). One week after the sclerotherapy, the platelet counts returned to (167-312) x 10(9)/L (average, (258.5 ± 34.4) x 10(9)/L). The hemoglobin and blood coagulation function returned to normal within 1-5 weeks. Meanwhile the mental condition, appetite, body weight, sleeping were greatly improved. The size of the lesions decreased gradually after the combined therapy including 13 cases within 3-12 months and 13 cases within 13-36 months. Long term follow-up indicated that only 1 case need treatment for recurrent decrease of platelet counts, and all of the 25 cases kept the normal weight, height, immunity as well as the growing development.

CONCLUSIONSOral corticosteroids plus intravenous vincristine combined with sclerotherapy is a reliable management with high cure rate, short course and minor side-effect.

Administration, Oral ; Betamethasone ; administration & dosage ; Combined Modality Therapy ; methods ; Ethanol ; administration & dosage ; Glucocorticoids ; administration & dosage ; Humans ; Infant ; Injections, Intravenous ; Kasabach-Merritt Syndrome ; blood ; therapy ; Platelet Count ; Polyethylene Glycols ; administration & dosage ; Retrospective Studies ; Sclerotherapy ; methods ; Vincristine ; administration & dosage

Hemangiomas are the most common benign tumors of the liver. They are generally asymptomatic, but giant hemangiomas can lead to abdominal discomfort, bleeding, or obstructive symptoms. Kasabach-Merritt syndrome is a rare but life-threatening complication of hemangioma, characterized by consumptive coagulopathy with large vascular tumors. More than 80% of Kasabach-Merritt syndrome cases occur within the first year of life. However, there are few reports of Kasabach-Merritt syndrome with giant hepatic hemangioma in adults and, as far as we know, no reports of Kasabach-Merritt syndrome with hepatic hemangioma treated with first line medical treatment only. The most important treatment for this syndrome is removal of the large vascular tumor. However, surgical treatment entails risk of bleeding, and the patient's condition can mitigate against surgery. We herein present a case of unresectable giant hepatic hemangioma with disseminated intravascular coagulopathy. The patient was a 60-year-old woman who complained of hematochezia, ecchymosis, and abdominal distension. She refused all surgical management and was therefore treated with systemic glucocorticoids and beta-blockers. After two weeks of steroid therapy, she responded partially to the treatment. Her laboratory findings and hematochezia improved. She was discharged on hospital day 33 and observed without signs of bleeding for three months.
Abdomen/diagnostic imaging ; Ecchymosis/etiology ; Female ; Gastrointestinal Hemorrhage/etiology ; Hemangioma/complications/*diagnosis ; Humans ; Kasabach-Merritt Syndrome/complications/*diagnosis/drug therapy ; Middle Aged ; Prednisone/therapeutic use ; Propranolol/therapeutic use ; Tomography, X-Ray Computed