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)

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

We describe a 23-year-old man with protein-losing enteropathy, iron deficiency anemia, and recurrent gastrointestinal bleeding. The patient eventually developed disseminated intravascular coagulation, portal vein thrombosis, and extensive small bowel infarction. The autopsy showed multiple telangiectases in the intestines, mesentery, liver, gallbladder, renal pelves, and diaphragm. In addition, lymphangiectases were found in the retroperitoneal space, intestines, and liver. These lesions appeared to have been the cause of the gastrointestinal bleeding and the protein-losing enteropathy. The case most likely represents Osler-Weber-Rendu disease without the usual manifestations in the skin and oral-nasal cavities.
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PMID:Multiple abdominal telangiectases and lymphangiectases. A limited form of Osler-Weber-Rendu disease? 858 83

A 45-year-old Japanese woman with splenomegaly and thrombocytopenia was referred to our hospital. The diagnosis of Osler-Weber-Rendu disease (Osler's disease) was made because of spotty telangiectasia on her tongue, recurrent epistaxis since childhood, and a diathesis indicated by her family history. The patient's laboratory examination revealed anemia, thrombocytopenia, and other data consistent with chronic disseminated intravascular coagulation (DIC). Bone marrow examination was normal. Abdominal computed tomography showed marked enlargement of the spleen with deformity and calcified plaque, not homogeneously enhancing. Hypersplenism was not observed. Platelet scintigraphy indicated a remarkable uptake in the spleen. She was diagnosed as having chronic DIC associated with vascular lesions of Osler's disease in the spleen. Splenectomy was performed and the subsequent pathological findings indicated that fragility of the fine vascular architecture of the splenic red pulp might have been responsible for pathogenesis. The large pooling of blood with coagulation was thought to be secondary.
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PMID:Splenomegaly and chronic disseminated intravascular coagulation in Osler-Weber-Rendu disease: a case report. 1107 62

Hereditary haemorrhagic telangiectasia (HHT) causes chronic nasal and gastrointestinal haemorrhage. Prothrombotic agents are commonly used for severe haemorrhage. Thrombotic risks have not been defined. In order to identify prothrombotic variables in HHT patients, and assess their potential functional significance, a pilot ELISA-based study comparing plasma proteins in healthy individuals with HHT to age/sex-matched non-HHT controls was validated in a full study of 309 consecutive HHT-affected individuals. In the pilot study, factor VIII (FVIII) and von Willebrand factor antigen concentrations were elevated in the HHT group compared to non-HHT controls (p<0.0013, Mann-Whitney). Service laboratory measurements confirmed high FVIII:Ag in 125 HHT-affected individuals with no recent ill-health, intervention or venous thromboemboli. FVIII:Ag levels increased with age. Logistic regression also suggested an age-independent association with HHT-associated pulmonary arteriovenous malformations (AVMs). No association was demonstrated between FVIII:Ag and acute phase response, disseminated intravascular coagulation, ABO group, pulmonary artery pressure, or markers of HHT haemorrhage. Elevated FVIII:Ag were associated with shortened activated partial thromboplastin times (APTTs), and VTE:VTE affected 20/309 (6.5%) HHT-affected individuals, at median age 61(36-71) years. Four VTE occurred in factorV Leiden heterozygotes in the months following PAVM-associated brain abscess. The strongest association with VTE was with log-transformed FVIII:Ag measured 10-132 months from VTE (odds ratio 2.41, 95% confidence intervals 1.254, 4.612, p=0.008). Age made no additional contribution to VTE risk once adjusted for FVIII:Ag. In conclusion, HHT-related elevation of FVIII:Ag levels may influence thrombotic risk in HHT. Individualised risk-benefit considerations may be helpful in HHT management.
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PMID:Elevated factor VIII in hereditary haemorrhagic telangiectasia (HHT): association with venous thromboembolism. 1861 56