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)

Multiorgan thrombotic disorders have been described in a variety of conditions including systemic lupus erythematosus. Among these are the 'catastrophic' antiphospholipid syndrome, disseminated intravascular coagulation and the thrombotic thrombocytopenic purpura-haemolytic uraemic syndrome. In this review we briefly analyse some specific clinical and haematological characteristics of these conditions and attempt to uncover common links between them.
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PMID:Multiorgan thrombotic disorders in systemic lupus erythematosus: a common link? 130 83

Purpura Fulminans and DIC were the main clinical manifestations of the antiphospholipid syndrome observed in a 62-year-old man. The patient was well until 44 years of age when he began to suffer from recurrent thrombophlebitis, without other symptoms suggestive of immune disease. At the time of hospital admission the pt. appeared acutely ill, showing high fever, severe anemia, massive urinary blood loss, multiple purpuric patches evolving to hemorrhagic bullae and gangrene rapidly spreading over about 30% of the total body area. No signs of neurological involvement or of visceral thrombotic occlusions were present. Clotting tests were consistent with a diagnosis of DIC, further confirmed by skin biopsy showing the presence of thrombi in dermal arterioles. The autoantibody research was positive as follows: Waaler-Rose 1:40, Anti-DNA 1:80; ANF 1:640, aCA IgG 100 GPL. LA was diagnosed according to standard criteria: prolonged KCT and RVVT not corrected by a mixture of normal plasma and abnormal TTI. Plasma exchange in association with heparin and prednisone was effective in arresting the progression of the skin lesion; nevertheless the patient died ten days after hospital admission for sepsi and acute renal failure.
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PMID:Purpura fulminans as clinical manifestation of atypical SLE with antiphospholipid antibodies: a case report. 180 49

A 34-year old woman, with a 3 yr history of severe seropositive rheumatoid arthritis (RA) with lupus anticoagulant and anticardiolipin antibodies, developed a massive anterior myocardial infarction and ischemia of the lower extremities, with disseminated intravascular coagulation resulting from extensive tissue damage. Seven days after admission, she died of severe heart failure complicated by ventricular fibrillation. To our knowledge, this is the first documented case of fatal acute antiphospholipid syndrome in RA.
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PMID:Catastrophic antiphospholipid syndrome with fatal acute course in rheumatoid arthritis. 747 89

We describe the case of a 25-year-old woman who presented with the antiphospholipid antibody syndrome (APS) manifesting as hemolytic anemia, thrombocytopenia, renal insufficiency, thromboses in multiple sites including skin, spleen and nasal mucosa, and multiple sites of avascular necrosis of bone. Interestingly, she also had low grade disseminated intravascular coagulation, which has been suggested to be a mechanism for avascular necrosis. We suggest that the APS may be one cause of thrombosis in avascular necrosis.
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PMID:Catastrophic antiphospholipid antibody syndrome presenting with multiple thromboses and sites of avascular necrosis. 769 46

We describe a patient with previous venous thrombosis while using oral contraceptives and recurrent pregnancy loss, who presented with massive hepatic infarction in the last trimester of the fourth gestation. Thrombocytopenia, the lupus anticoagulant (LA) and the anticardiolipin antibody (aCL) were detected and a diagnosis of a 'primary' antiphospholipid syndrome (APS) was made. The clinical and histological manifestations and the differential diagnosis, especially with DIC and pre-eclampsia, are discussed.
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PMID:Hepatic infarction in a pregnant patient with the 'primary' antiphospholipid syndrome. 826 78

A 27-year-old primipara with severe preeclampsia and primary antiphospholipid syndrome developed right upper quadrant pain, massive ascites, HELLP syndrome, and disseminated intravascular coagulation shortly following vaginal delivery. Computed tomography and color Doppler studies were compatible with complete thrombosis of the right hepatic veins, the Budd-Chiari syndrome. Anticoagulation was initiated, along with supportive measures, and the patient recovered completely. Imaging studies 6 months later were normal. This case demonstrates that nearly fatal forms of venous thrombosis may complicate preeclampsia in women with antiphospholipid syndrome; Doppler studies of the hepatic vein are of value in establishing the diagnosis.
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PMID:Budd-Chiari syndrome complicating severe preeclampsia in a parturient with primary antiphospholipid syndrome. 888 13

Thirty one patients with antiphospholipid antibodies who developed multi-organ failure ("Catastrophic Antiphospholipid Syndrome") are reviewed. Thirteen suffered from a 'Primary' antiphospholipid syndrome, 13 from defined SLE, 4 from 'lupus-like' disease and one from rheumatoid arthritis. In more than one third precipitating factors were evident (e.g. infections, major/minor surgical procedures, oral contraceptives). Death occurred in 60% of patients from a variety of causes (myocardial failure, ARDS, CNS causes or, often a combination). Disseminated Intravascular Coagulation was present in 8 of 31 patients. Plasmapheresis appeared to be useful in several who had not responded to conventional therapy (e.g. IV heparin, steroids, immunosuppression).
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PMID:The catastrophic antiphospholipid syndrome 1996: acute multi-organ failure associated with antiphospholipid antibodies: a review of 31 patients. 890 72

Catastrophic antiphospholipid antibody syndrome, reported in a minority of patients with circulating antiphospholipid antibodies, is characterized by widespread vascular occlusions. The term "catastrophic" has been used to describe the severity of symptomatology, sometimes leading to death. We describe a girl aged 11 years, fulfilling diagnostic criteria for systemic lupus erythematosus, with recurrent episodes of thromboembolic phenomena involving lung and skin, complicated with disseminated intravascular coagulation. Treatment with warfarin ultimately resulted in effective control of the disease.
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PMID:Catastrophic antiphospholipid antibody syndrome in pediatric systemic lupus erythematosus. 903 2

A 41-year-old woman with complete protein S (PS) deficiency who developed diffuse proliferative lupus nephritis is reported. She was referred to our hospital with nephrotic syndrome and thrombocytopenia. Her medical history included colorectostomy and amputation of the extremities because of repeated thrombotic episodes during her teens without any evidence of systemic lupus erythematosus. The diagnosis of PS deficiency was made from the patient's clinical course, undetectable serum PS in either the active or inactive form, normal protein C activity, and no evidence of the antiphospholipid syndrome. However, there was no definitive family history. A depressed level of complements and a positive antinuclear acid antibody suggested a diagnosis of systemic lupus erythematosus. The patient had a rapidly progressive course and died of disseminated intravascular coagulation. An autopsy showed generalized thrombotic lesions and diffuse proliferative lupus nephritis on both ordinal light and immunoperoxidase microscopy. Our observations suggest that PS-deficient patients may have a hyperinflammatory response.
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PMID:A 41-year-old woman with protein S deficiency and diffuse proliferative lupus nephritis: is protein S deficiency associated with a hyperinflammatory response? 918 80

Blood coagulation tests are useful to diagnose some thrombotic diseases. Particularly, these tests are valuable for the diagnosis of familiar thrombophilia, antiphospholipid antibody syndrome (APS) and disseminated intravascular coagulation (DIC). For the diagnosis of thrombophilia, determinations of both biological activity and antigen level of antithrombin III, protein C and protein S are important for initial screening. Since activated protein C (APC) resistance is extremely rare in Japanese, APC resistant test that based on APTT, is unnecessary to include as one of the screening tests. Detection of activity and antigen level of either plasminogen or fibrinogen is recommended to screen the plasminogen deficiency or dysfibrinogenemia. Determination of lupus anticoagulant is needed for the diagnosis of APS. At this time, the dilute phospholipid APTT (dAPTT) or the dilute Russell viper venom time (dRVVT) may be useful as a screening test for LA because procedure of these tests are basically simple to perform in Japanese laboratory. In the next step, cross mixing test of dAPTT (or APTT) should be perform to make a diagnose of LA more solid. Final confirm tests can be conveniently carried out with kit of either STACLOT or LA-CONFIRM. Platelet count and FDP (or FDP D dimer) assay are two essential tests for the diagnosis of DIC. Criteria of diagnosis for DIC recommended by Blood Coagulation Research Group of Japanese Ministry of Health and Welfare is not unnecessarily appropriate for practical use. TAT and PIC can be a good laboratory tests for early detection of hypercoagulable state in patients with DIC.
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PMID:[Clinical diagnosis of thrombosis and blood coagulation tests]. 956 63


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