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)

A review of 250 patients with the catastrophic antiphospholipid (Asherson's) syndrome (CAPS) taken from the web site organized by the Europhospholipid Group (http://www.med.ub.es/MIMMUN/FORUM/CAPS.HTM) is presented in this paper. A short historical overview of the antiphospholipid syndrome (APS) is followed by a description of the "triggering" factors, associated autoimmune diseases, clinical presentation, presumed pathogenesis, prognosis, mode of death and suggested therapies. Triggering factors are present in approximately 50% of patients and consist predominantly of infections, trauma, including minor surgical procedures such as biopsies, obstetric-related multiorgan failure and malignancy-associated CAPS. The patients present mainly with multiorgan failure resulting from predominantly small vessel occlusions affecting mainly intra-abdominal organs such as bowel, liver, pancreas, and adrenals, although large vessel occlusions do occur and comprise mainly deep vein thromboses (DVT) of the veins of the lower limbs and arterial occlusions causing strokes and peripheral gangrene. They do not however dominate the clinical picture. The condition differs considerably from the simple/classic APS in several respects, viz. the rapid development of multiorgan failure following the above-mentioned identifiable precipitating factors, the involvement of unusual organs such as bowel, reproductive organs, and bone marrow, complicating features of disseminated intravascular coagulation in 20% of cases, the acute (adult) respiratory distress syndrome (ARDS) in one third of patients, and severe thrombocytopenia; these not being encountered in the simple/classic APS. Treatment consisting of regular and repeated plasma exchanges using fresh frozen plasma, and IV immunoglobulins in addition to parenteral steroids and anticoagulation are necessary to improve the survival in a condition where the mortality is still of the order of 50%. Treatment may have to be continued for several weeks. Parenteral antibiotics may be indicated where an underlying infection is suspected. Antifungal therapy may also be indicated with prolonged treatment and the use of the monoclonal anti-CD20 molecule, Rituximab, has proven useful in those patients where thrombocytopenia poses a major risk of hemorrhage.
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PMID:Multiorgan failure and antiphospholipid antibodies: the catastrophic antiphospholipid (Asherson's) syndrome. 1632 90

The thrombotic thrombocytopenic purpura syndrome (TTP) can be mistaken for a number of other conditions, and it is important to diagnose correctly and treat appropriately. We describe the features of TTP that can help make a positive diagnosis and other conditions in the differential diagnosis with symptoms that can overlap and mimic those of TTR. We discuss TTP and its variants, hemolytic uremic syndrome, disseminated intravascular coagulation, heparin-induced thrombocytopenia, antiphospholipid syndrome, Evans syndrome, preeclampsia/eclampsia, HELLP syndrome, acute fatty liver of pregnancy, and multiorgan failure.
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PMID:Thrombotic thrombocytopenic purpura and its look-alikes. 1649 32

Hematologic abnormalities may occur in antiphospholipid antibody syndrome. The most common of these abnormalities is thrombocytopenia. The mechanism by which low platelet counts occur is not known but studies continue to elucidate the potential role of autoantibodies and cell surface markers. The risk for thrombosis in patients with thrombocytopenia does not appear to be related to the presence of thrombocytopenia itself. Innovative treatment with anti-CD20 antibodies may be of benefit in reversing thrombocytopenia and treatment with splenectomy does not appear to increase the risk for subsequent thrombosis. Hemolytic anemia is associated with antiphospholipid antibodies as well, but the risk for thrombosis in patients with hemolytic anemia is not clearly elevated. Diagnostic features of disseminated intravascular coagulation may co-occur with the antiphospholipid syndrome and may be associated with a catastrophic clinical presentation.
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PMID:Prevalence of secondary hematologic disorders in the antiphospholipid syndrome: impact on coagulation risk. 1656 67

The catastrophic variant is an accelerated form of the antiphospholipid syndrome resulting in multiorgan failure because of multiple small vessel occlusions. We report a case of catastrophic antiphospholipid syndrome in a patient with subacute cutaneous lupus erythematosus and ischemic bowel, who presented with acute abdominal pain due to diffuse right colon and small bowel necrosis requiring large resection, associated with acute respiratory distress syndrome, thrombocytopenia and disseminated intravascular coagulation. Histopathological examination of resected tissues showed diffuse arteriolar and venous thrombosis but no vasculitis, and mesenteric artery lumen severely narrowed by intimal fibrosis. The patient died 15 days after admission despite treatment with anticoagulation, steroids, continuous hemofiltration and plasma exchange. Ischemic bowel and diffuse intestinal necrosis may be secondary to the antiphospholipid syndrome, and a high level of suspicion and an early diagnosis are required.
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PMID:Diffuse large and small bowel necrosis in catastrophic antiphospholipid syndrome. 1689 16

The concept of "probable" antiphospholipid syndrome (APS) is almost identical with several conditions which may presage the development of the APS with its major complications of large vessel thromboses resulting in deep vein occlusions in the lower limbs (DVT) particularly and strokes. These conditions comprising livedo reticularis, chorea, thrombocytopenia, fetal loss and valve lesions. These conditions, comprising livedo reticularis, chorea, thrombocytopenia, fetal loss and valve lesions may be followed, often years later by diagnosable APS. The issue whether these patients should be more aggressively treated on presentation in order to prevent the thrombotic complications. A new subset of the APS is proposed viz. microangiopathic antiphospholipid syndrome ("MAPS") comprising those patients presenting with thrombotic microangiopathy and demonstrable antiphospholipid antibodies who may share common although not identical provoking factors (e.g. infections, drugs), clinical manifestations and haematological manifestations (severe thrombocytopenia, hemolytic anaemia) and treatments viz. plasma exchange. Patients without large vessel occlusions may be included in the MAPS subset. These conditions include thrombotic thrombocytopenic purpura (TTP), hemolytic-uremic syndrome (HUS), and the HELLP syndrome. Patients with catastrophic antiphospholipid syndrome (CAPS) who do not demonstrate large vessel occlusions also fall into this group. Disseminated intravascular coagulation (DIC) has also been reported with demonstrable antiphospholipid antibodies and also manifests severe thrombocytopenia and small vessel occlusions. It may cause problems in differential diagnosis.
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PMID:New subsets of the antiphospholipid syndrome in 2006: "PRE-APS" (probable APS) and microangiopathic antiphospholipid syndromes ("MAPS"). 1713 47

The association between thrombosis and cancer has been extensively studied since first pointed out by Trousseau in 1895. It is, however, not commonly appreciated that the incidence of thrombosis in malignant hematologic disorders is as high or even higher than in solid tumors. Thrombotic complications in acute leukemia are often overlooked because bleeding complications generally dominate the clinical picture. Yet, the patient is at risk for both. While there are many thrombogenic factors shared by both solid tumors and leukemia, many additional prothrombotic features are present in leukemia. The prothrombotic factors include hyperleukocytosis, increased expression of tissue factor and its activation in leukemic cells, and the prothrombotic adverse effects of therapeutic agents and vascular access catheters. In addition, comorbid conditions including hereditary thrombophilia, infection, endothelial cell activation by cytokines, antiphospholipid syndrome and acquired activated protein C resistance are major contributory factors. Factors that increase the bleeding risk include thrombocytopenia, disseminated intravascular coagulation, and excessive fibrinolysis, which is enhanced by increased expression of Annexin II by leukemic cells. Therapeutic approaches to both bleeding and thrombotic conditions require special considerations of these factors.
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PMID:Double hazard of thrombophilia and bleeding in leukemia. 1802 23

Catastrophic antiphospholipid syndrome (CAPS), described by Asherson in 1992, is a rare form of antiphospholipid syndrome resulting in multiorgan failure with a mortality rate of about 50%. The syndrome occurs in patients with either systemic lupus erythematosus and other rheumatic diseases (systemic sclerosis, rheumatoid arthritis, primary Sjogren syndrome) or alone. Whereas in "classic" antiphospholipid syndrome (APS), medium-large vessels are involved, a diffuse small vessel ischemia and thrombosis (microangiopathic disease) leading to a severe multiorgan dysfunction is predominant in CAPS. "Trigger" factors have been demonstrated in 45% of patients, but in the majority, they remain unknown. Not infrequently, CAPS arises in patients without any previous thrombotic history. The kidney is the organ most commonly affected, followed by the lung, the central nervous system, the heart and the skin. Disseminated intravascular coagulation occurs in approximately 13% of patients. The present study reports the clinical and serological features of 4 patients affected by this rare form of antiphospholipid syndrome. Nephrologists should be aware of the possibility of this syndrome as a cause of multiorgan failure since prompt recognition is essential for effective treatment.
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PMID:Catastrophic antiphospholipid syndrome: report of 4 cases. 1804 77

The catastrophic antiphospholipid syndrome is a rare and devastating manifestation of the antiphospholipid syndrome. It is associated with acute multi-organ failure involving three or more end organs in patients with antiphospholipid antibodies. A precipitating event can be found in some cases, and mortality rates have approached 60%. This article describes a case of catastrophic antiphospholipid syndrome in a patient who presented with Budd-Chiari syndrome from membranous obstruction of the inferior vena cava and extensive bone marrow necrosis shortly after a surgical procedure. These and other clinical manifestations of multi-organ failure (including low-grade disseminated intravascular coagulation) were associated with high titers of immunoglobulin M anticardiolipin antibodies. The patient fully recovered after being treated with percutaneous transluminal angioplasty and aggressive anticoagulation. The pathogenesis of catastrophic antiphospholipid syndrome in this patient may well have resulted from in situ thrombus formation around an intravascular coagulation web (congenital or acquired) from a patient predisposed to a hypercoagulable state from anticardiolipin antibodies and a recent surgical procedure.
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PMID:Membranous obstruction of the inferior vena cava and extensive bone marrow necrosis associated with catastrophic antiphospholipid syndrome. 1907 9

In hematological disorders, thrombocytopenia is frequently observed, and it is sometimes difficult to diagnose the underlying disease. In this symposium, laboratory tests for platelet abnormality were reviewed. Tests for platelet aggregation were reported to be important for the diagnosis of platelet dysfunction. Thrombocytopenia is caused by disseminated intravascular coagulation (DIC), thrombotic microangiopathy (TMA), heparin-induced thrombocytopenia (HIT), antiphospholipid syndrome (APS), idiopathic thrombocytopenic purpura (ITP), etc. As DIC is classified according to the degree of fibrinolysis, it was stated that the measurement of hemostatic molecular markers was further required. TMA is caused by abnormality of ADAMTS13, verotoxin, DIC, etc. HIT is diagnosed by anti-PF4 antibody, but its specificity is not high. Further investigation of TMA and HIT is required. APS is one of the most important diseases which cause thrombosis or abortion, suggesting that a differential diagnosis of APS is important. It was reported that diagnostic criteria of ITP have been established using a new antibody assay for platelets, immature platelet fractions, thrombopoietin, etc. In myeloproliferative disorders such as polycythemia vera and essential thrombocythemia, the mutation of JAK2 V617F was reported to be an important risk factor for thrombosis.
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PMID:[Summary of pathophysiology and diagnosis of patients with platelet abnormality]. 1952 56

An immune-mediated, severe, acquired prothrombotic disorder, heparin-induced thrombocytopenia type II (HIT II) occurs in 0.5-5% of patients exposed to unfractionated heparin longer than 5-7 days. Arterial and venous thromboses are induced by HIT II in about 35-50% of patients. Typical death rate for HIT is about 29%, while 21% of HIT patients result in amputation of a limb. The trend towards the occurrence of HIT due to the administration of low molecular weight heparins (LMWH) taking ever conspicuous place in the standard venous thromboembolism (VTE) prophylaxis has been more frequently observed recently. It is considered that LMWH may cause HIT II in about 0.25-1%. The need for further modification of HIPA assays with LMWH has been imposed in the HIT laboratory diagnostics, heretofore overburdened with complexity. There are several constantly opposing problems arising in HIT laboratory diagnostics, one of which is that in a certain number of patients immunologic assays detect nonpathogenic antibodies (mainly IgM or IgA heparin-PF4 antibodies) while, on the other hand, the occurrence of HIT pathogenetically mediated by minor antigens (neutrophil-activating peptide 2 or interleukin 8) may be neglected in certain cases. The following factors play an important role in the interpretation of each laboratory HIT assays performed: 1. correlation with HIT clinical probability test, the best known of which is 4T'score, 2. the interpretation of the laboratory findings dependent on the time of the thrombocytopenia onset, as well as 3. the sensitivity and specificity of each test respectively. The HIT diagnostics in the presence of other comorbid states which may also induce thrombocytopenia, more precisely known as pseudo HIT (cancer, sepsis, disseminated intravascular coagulation, pulmonary embolism, antiphospholipid syndrome, etc), represents a specific clinical problem.
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PMID:Obstacles in the diagnostics and therapy of heparin-induced thrombocytopenia. 2022 87


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