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Query: UMLS:C0012739 (disseminated intravascular coagulation)
 8,673 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Hemangiomas are the most frequent tumors found in newborns, but mostly regress by the age of 7. Spontaneous progression and such complications as hemorrhage, disseminated intravascular coagulation (Kasabach-Merritt syndrome), threatened cardiovascular decompensation, and threatened interference with vital functions are rare and must be treated. Embolization therapy is a new therapeutic approach: vascular malformations and hemangiomas with pronounced arteriovenous shunts are treated by selective arterial embolization of the most peripheral branches of the supplying blood vessels. In contrast, lesions with a steady, slow blood perfusion can be treated by direct embolization of the cavernous system; further thrombosis and organization of the thrombus may lead to a permanent and complete occlusion of the hemangioma. If permanent occlusion cannot be achieved, temporary or partial thrombosis may allow curative plastic or reconstructive surgery, which would otherwise be impossible.
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PMID:[Embolization therapy of cavernous hemangiomas]. 279 61

The Kasabach Merritt syndrome consists of thrombocytopenia, microangiopathic hemolytic anemia, and a localized consumption coagulopathy that develops within the abnormal vascular channels of a hemangioma. In general, these patients demonstrate only mild abnormalities of screening clotting tests, but they can potentially develop life-threatening complications. We present a patient who developed a severe anemia that was refractory to erythrocyte transfusions. Treatment with epsilon-aminocaproic acid to inhibit fibrinolysis and cryoprecipitate to replenish his deficient circulating fibrinogen interrupted the cycle of his systemic coagulopathy and enabled us to transfuse him to a normal hematocrit.
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PMID:Antifibrinolytic therapy in the management of the Kasabach Merritt syndrome. 274 17

Kasabach-Merritt syndrome with consumption coagulopathy occurred in a 5-week-old female infant with a hemangioma on her back. Disseminated intravascular coagulopathy was improved by an exchange transfusion. Administration of steroid and radiation therapy was attempted as a treatment for the hemangioma and thrombocytopenia; however, it was unsuccessful. Complete recovery was obtained by surgical excision. Enhanced computerized tomography was advantageous to determine the size of the hemangioma prior to operation.
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PMID:Kasabach-Merritt syndrome with disseminated intravascular coagulopathy treated by exchange transfusion and surgical excision. 379 4

Kasabach-Merritt syndrome consists of thrombocytopenia, microangiopathic hemolytic anemia, and an acute or chronic consumption coagulopathy in association with a rapidly enlarging hemangioma. Although the potential for serious morbidity is great, the condition remits when the hemangioma begins to involute. Four infants with large congenital cavernous hemangiomas and Kasabach-Merritt syndrome are described. All four received oral prednisone for variable periods; two received aspirin and one dipyridamole. Two patients required intravenous heparin because of life-threatening disseminated intravascular coagulation and bleeding. Two patients experienced almost total involution of their hemangiomas by the age of 2 years, and one by the age of 13 months. The fourth patient showed signs of beginning resolution of the hemangioma at the age of 8 months and hematologic values returned to normal levels. No side effects from therapy were noted in any of these infants.
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PMID:Kasabach-Merritt syndrome in infants. 685 83

In summary, 47 patients with documented HHT and a bleeding problem were referred to San Joaquin Hematology Oncology Medical Group over a 2-year period. Fifty-one percent of patients were noted to have an associated DIC syndrome and of these 24 patients, 19 had acute DIC episodes, six had chronic DIC, six presented with diffuse, recurrent deep venous thrombosis and of these six, three suffered pulmonary emboli. Other defects were also noted and were thought to be coincidental defects. This syndrome should be readily considered and searched for when seeing patients with HHT, especially if significant hemorrhage or thrombosis is present. It should further be appreciated that many patients with HHT and bleeding are candidates for the development of acute or chronic DIC and thus a "mini" Kasabach-Merritt syndrome. When patients with HHT present with undue bleeding, this syndrome should be appreciated, searched for from the clinical and laboratory standpoint and, when found, treated in the appropriate manner with supportive therapy, mini-heparin or antiplatelet therapy as the clinical situation dictates.
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PMID:Hereditary hemorrhagic telangiectasia and disseminated intravascular coagulation: a new clinical syndrome. 697 47

The Kasabach-Merritt syndrome is the association of cavernous hemangiomas and consumption coagulopathy marked by anemia, thrombocytopenia, and hypofibronigenemia. Exacerbation of the consumption coagulopathy has been described in the 2 previous reports of this syndrome when associated with pregnancy. The authors report a third patient whose delivery and postopartum course were marked by increased coagulation abnormalities and subsequent hemorrhage. This patient's 32-day hospital course and need for multiple blood transfusions, clotting factors, platelets, heparin, and finally epsilon-aminocaproic acid underscore the need for patients with this syndrome to deliver in a referral center hospital where replacement therapy and hematologic consultation are readily available.
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PMID:Pregnancy complicated by the Kasabach-Merritt syndrome. 719 Feb 57

The Kasabach-Merritt syndrome of consumptive coagulopathy associated with massive hemangiomas is a potentially life-threatening problem in patients who sustain long bone fractures of the involved extremities. In this syndrome, platelet consumption is caused by their sequestration in the sinusoids and epitheloid chambers of large hemangiomas. Secondary fibrinolysis then occurs with resulting thrombocytopenia, hypofibrinogenemia, and increased fibrin degradation products that can lead to disseminated intravascular coagulation. This can result in massive bleeding even after minor trauma. In such patients, operative management of long bone fractures, including the placement of cutaneous pins for skeletal traction, may be contraindicated; nonoperative management may be necessary. Kasabach-Merritt syndrome must be suspected in patients with large hemangiomas and associated long bone fractures, and appropriate coagulation studies should be obtained before operative management or placement of percutaneous skeletal pins. Decreased hematocrit and fibrinogen levels associated with thrombocytopenia and prolonged prothrombin time and partial thromboplastin time should alert the orthopaedist to the possibility of Kasabach-Merritt syndrome, and prompt hematologic consultation should be obtained. If surgical treatment is deemed too dangerous because of the possibility of uncontrollable disseminated intravascular coagulation, the only prudent option may be a closed reduction and cast application after appropriate medical management of coagulation parameters.
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PMID:Kasabach-Merritt syndrome complicating treatment of a closed femoral fracture. 763 3

A 20 year-old female patient with Kasabach-Merrit syndrome, suffered from chronic consumption coagulopathy due to localized intravascular coagulation in the tumors. She had been diagnosed as Kasabach-Merrit syndrome immediately after birth and below knee amputation of her right lower leg was performed at the age of 2 years because of her giant hemangioma on the right foot and lower leg. After the operation, she had often complained of severe pain and enlargement of the residual tumors due to continuous thrombus formation within the tumors. She was admitted to the third Department of Tohoku University Hospital in order to initiate oral anticoagulant therapy with Warfarin at the age of 12 years. After the administration of 2.5 mg/day Warfarin, she has maintained good clinical condition until now, despite the occasional occurrence of coagulation abnormalities. We believe that the results of this case indicate the efficacy of oral anticoagulant therapy in the treatment of chronic consumption coagulopathies complicated with other diseases.
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PMID:[A case of Kasabach-Merrit syndrome complicated with DIC treated effectively by long term oral administration of warfarin]. 778 22

We describe the case of a 42-year-old woman with giant cavernous hemangioma and Kasabach-Merritt syndrome. The patient presented with consumption coagulopathy due to intravascular, intratumoral coagulation as revealed by low platelet levels, fibrinogenopenia and an increase in fibrinolysis with high levels of fibrinogen degradation products. She was scheduled to receive an orthotopic liver transplant because of three factors: respiratory distress caused by compression of the diaphragm by the giant tumor; the risk of bleeding caused by spontaneous rupture or trauma; and the presence of Kasabach-Merritt syndrome due to consumption coagulopathy. Before surgery fibrinogen deficit was corrected with 4 units of cryoprecipitates and low platelet level was treated with 10 units of platelets. Coagulopathy during surgery was corrected with fresh plasma (17 units), cryoprecipitates (6 U), aprotinin (1 x 10(6) U/kg) and antithrombin 3 (2000 U). Blood loss was compensated for with 9 units of packed red blood cells. This report describes the procedures used for anesthesia, for prevention of accidental bleeding during surgery, hemodynamic control and preoperative coagulation testing.
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PMID:[Orthotopic liver transplant for giant cavernous hemangioma and Kasabach-Merritt syndrome]. 789 56

Giant vascular neoplasms in neonates generally require aggressive medical or surgical therapy for treatment of complications. Steroids, chemotherapy, embolization, radiation, and surgery have all been used with short-term beneficial and sometimes unknown long-term side effects. A new modality of treatment, alpha-interferon, has recently been described. The majority of hemangiomas in children involute by 8 years of age. Occasionally, hemangiomas can endanger vital structures and are associated with a consumption coagulopathy and thrombocytopenia (Kasabach-Merritt Syndrome). These hemangiomas occasionally do not respond to steroids, radiation therapy, cytotoxic drugs, or embolization. The mortality rates approach 50% in nonresponders. Alpha-interferon has been used in these children with life-threatening complications of hemangiomas with relief of symptoms. This case illustrates the potential use of alpha-interferon in the management of giant hemangiomas in children. This emerging form of biological therapy avoids the risks of radiation therapy, embolization, and surgery with only minimal side effects.
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PMID:Successful management of an infant with a giant hemangioma of the retroperitoneum and Kasabach-Merritt syndrome with alpha-interferon. 826 1


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