Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0012739 (disseminated intravascular coagulation)
 8,673 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Confusion in the nomenclature of vascular malformations has been a major obstacle to the understanding of these conditions, so that misdiagnosis and treatment inconsistencies are common. Coagulation abnormalities occurring in combination with venous malformations (VM) have been misdiagnosed as Kasabach-Merritt syndrome (KMS), despite marked differences in clinical features, pathology and treatment. A homogenous group of 24 patients with diffuse limb VM was entered into a retrospective chart review study. The VM affected an upper limb in 12 patients, a lower limb in 10 and both in two. Localized intravascular coagulation (LIC) was characterized by a decrease in fibrinogen (0.5-1 g/l), an increase in d-dimers (2-64 micro g/ml) and presence of soluble complex of fibrin (+ to +++). Platelet counts were normal or slightly decreased. Higher VM severity scores were associated with more severe LIC. A number of events such as sclerotherapy, surgery, bone fracture, prolonged immobilization and pregnancy or menstruation triggered conversion of the LIC to disseminated intravascular coagulation (DIC), with bleeding related to factor consumption and multiorgan failure related to disseminated microvascular thrombosis. Clinical symptoms associated with worsening of LIC were pain, thrombosis and bleeding at wound sites or during surgery. None of the patients had the large ecchymotic and inflammatory tumours seen in KMS. Graded permanent elastic compression with heparin therapy was the only effective treatment. In conclusion, VM-associated LIC is a distinctive lifelong coagulopathy that must be differentiated from KMS, which is characterized by platelet trapping within a vascular tumour of infancy. The treatment of the two conditions is very different.
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PMID:Coagulation abnormalities associated with extensive venous malformations of the limbs: differentiation from Kasabach-Merritt syndrome. 1218 Oct 29

Epithelioid hemangioendothelioma of the liver is a rare vascular neoplasm with intermediate malignant potential. The prognosis is highly unpredictable. We report the case of a 59-year-old woman who had the tumor radically resected, but multiple metastases of the liver developed associated with thrombocytopenia and consumption coagulopathy, as observed in Kasabach-Merritt syndrome. The patient did not respond to any treatment and the behavior of the tumor was very aggressive. The patient died 15 months after radical resection of the tumor.
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PMID:Epithelioid hemangioendothelioma of the liver associated with thrombocytopenia and coagulopathy. 1239 62

Diffuse haemangioma and intra-abdominal lymphangioma are rare in adults. In this case report, we present a 33-year-old female with coexisting multiple cutaneous and visceral cavernous haemangiomas and two huge intra-abdominal lymphangiomas of 25 and 35 cm in diameter. The organs involved were the liver, pericardium, renal hilus and bladder. She died due to disseminated intravascular coagulation and multiorgan failure, which resembled Kasabach-Merritt syndrome. The coexistence of generalised haemangiomas and intra-abdominal lymphangiomas and the lack of complaints until the age of 33 years makes her an unusual case in the literature. We also emphasise the other clinical conditions that should be considered in the differential diagnosis.
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PMID:Coexistence of cystic intra-abdominal lymphangiomas and diffuse venous haemangiomas in adult life. 1276 32

The Fps/Fes tyrosine kinase has been implicated in the regulation of hematopoiesis and inflammation. Mice expressing an activated variant of Fps/Fes (MFps) encoded by a gain-of-function mutant transgenic fps/fes allele (fps(MF)) exhibited hematological phenotypes, which suggested that Fps/Fes can direct hematopoietic lineage output. These mice also displayed marked hypervascularity and multifocal-hemangiomas which implicated this kinase in the regulation of angiogenesis. Here we explored the potential involvement of Fps/Fes in the regulation of hemostasis through effects on blood cells and the vascular endothelium. Hematological parameters of fps(MF) mice were characterized by peripheral blood analysis, histology, and transmission electron microscopy. Hemostasis parameters and platelet functions were assessed by flow cytometry and measurements of activated partial thromboplastin time, prothrombin time, thrombin clot time, platelet aggregation, bleeding times and in vitro fibrinolytic assays. Hematological and morphological analyses showed that fps(MF) mice displayed mild thrombocytopenia, anemia, red cell abnormalities and numerous hemostatic defects, including hypofibrinogenemia, hyper-fibrinolysis, impaired whole blood aggregation and a mild bleeding diathesis. fps(MF) mice displayed a complex array of hemostatic perturbations which are reminiscent of hemostatic disorders such as disseminated intravascular coagulation (DIC) and of hemangioma-associated pathologies such as Kasabach-Merritt phenomenon (KMS). These studies suggest that Fps/Fes influences both angiogenic and hemostatic function through regulatory effects on the endothelium.
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PMID:Hemostatic and hematological abnormalities in gain-of-function fps/fes transgenic mice are associated with the angiogenic phenotype. 1555 33

Primary angiosarcoma of the breast is a rare aggressive tumour of unknown etiology. A frequent clinical presentation is a painful palpable smooth mass or diffuse enlargement of the breast without a palpable mass but with purple discolouration of the overlying skin with apparent bruising. An uncommon clinical presentation of an angiosarcoma is spontaneous bleeding due to disseminated intravascular coagulation (DIC) by consumption coagulopathy, known as the Kasabach-Merritt syndrome. Imaging characteristics of a breast angiosarcoma are limited to a few radiological reports. We report a case of a young pregnant woman with a bleeding angiosarcoma of the breast and associated Kasabach-Merritt syndrome and describe the sonographic and MRI findings.
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PMID:Primary angiosarcoma of the breast associated Kasabach-Merritt syndrome during pregnancy. 1600 Feb 50

n patients with Kasabach-Merritt syndrome (KMS), local activation of coagulation commonly results in disseminated intravascular coagulation (DIC). Progress of DIC is associated with 30-40% mortality as a result of uncontrollable hemorrhage. A 39-year-old woman with an enlarging giant liver hemangioma was diagnosed as having KMS with DIC. To control the hemorrhagic diathesis, we commenced combination therapy for DIC with danaparoid (1,250 Ux2/day, intravenously (IV)) and tranexamic acid (0.5 g x 3/day, peros (PO). Rapid improvement of the bleeding tendency and coagulopathy occurred in response to this treatment - that is, DIC was controlled without removing the giant hemangioma. The therapy did not restrict the behavior of the patient by continuous drip and angiography could be performed without bleeding. Such therapy may be beneficial in chronic DIC with activation of fibrinolysis.
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PMID:Kasabach-Merritt syndrome associated with giant liver hemangioma: the effect of combined therapy with danaparoid sodium and tranexamic acid. 1626 20

Kasabach-Merritt Syndrome (KMS) originally referred to a hemangioma associated with life-threatening thrombocytopenia and a consumption coagulopathy. Treatment includes surgery, steroids, and alpha interferon. However response may be unsatisfactory and mortality rates of 10-37% have been reported. We report a case of a 4-month-old male whose disease had not responded to steroids and alpha interferon. He required ventilation, and his condition was deteriorating. It was thought that he would probably die of his condition. He was treated with 9 Gy in three fractions weekly of radiotherapy (RT). Clinical response was good and he is well and free from progression 4 years and 7 months after RT. A review of the literature on the role of RT in the management of hemangiomas is presented.
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PMID:Radiotherapy for life-threatening mediastinal hemangioma with Kasabach-Merritt syndrome. 1645 98

A male neonate was born with a large blue-purple swelling at the upper left leg. This was accompanied by a severe thrombocytopenia, a microangiopathic hemolytic anemia and a consumption coagulopathy: Kasabach Merritt syndrome.
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PMID:[Diagnostic image (322). A neonate with a swelling on one leg. Kasabach Merritt syndrome]. 1750 85

Proteus syndrome is a rare hamartomous disorder with highly variable manifestations of disproportionate overgrowth of body parts, epidermal nevi and vascular malformations. We describe two patients who had typical clinical and imaging Proteus syndrome features. One of most characteristic findings in these two patients was that they had giant hemangiomas of the spleen that caused Kasabach-Merritt syndrome, demonstrating clinical bleeding and disseminated intravascular coagulation laboratory alterations. The present two cases are the first report of this complication in Proteus syndrome. The patients' bleeding tendency and hemostatic defects were completely corrected after they successfully underwent splenectomy.
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PMID:Kasabach-Merritt syndrome caused by giant hemangiomas of the spleen in patients with Proteus syndrome. 1758 27

Here we report a patient with stage-4 bilateral cystic adrenal neuroblastomas with disseminated liver metastases and consumption coagulopathy who underwent liver transplant. Our patient was initially diagnosed with infantile hepatic hemangioendothelioma and bleeding into the adrenal glands secondary to consumption coagulopathy (Kasabach-Merritt syndrome). Liver transplant was performed as a life-saving procedure under this diagnosis. We discuss this unique patient because of the diagnostic pitfalls of this rare disease and the successful clinical outcome after LT and subsequent chemotherapy for neuroblastoma.
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PMID:Liver transplant in an infant with bilateral cystic neuroblastoma complicated by hepatic metastases and life-threatening consumption coagulopathy. 1833 88


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