Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0024141 (systemic lupus erythematosus)
 44,322 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Varicella is not always a benign disease it may cause serious complications. We report a two-year-old boy with disseminated intravascular coagulation in association with varicella. The patient had the lupus anticoagulant, the antiphospholipid antibody, acquired free protein S deficiency, and increased concentrations of the prothrombin F 1+2 fragment. Intravenous immunoglobulin was administered due to its potential antibody-blocking activity, and the patient responded well. We recommend that children with varicella and disseminated intravascular coagulation should be examined for the lupus anticoagulant, the free protein S antigen, the prothrombin fragment F 1+2 and the other coagulation parameters. Intravenous immunoglobulin administration could be useful in such conditions because of its antibody-blocking activity.
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PMID:Lupus anticoagulant and protein S deficiency in a child who developed disseminated intravascular coagulation in association with varicella. 1143 93

We retrospectively analyzed 52 adult patients with hemophagocytic syndrome (HPS). The underlying diseases were heterogeneous, including malignant lymphoma (lymphoma-associated hemophagocytic syndrome [LAHS]) in 26 patients, systemic lupus erythematosus in 3 patients, viral infections in 7 patients, and bacteria] or fungal infections in 6 patients. More than 83% of patients received prednisolone as an initial treatment. Multiple-agent chemotherapies (cyclophosphamide, doxorubicin, and vincristine) were administered to 96% of LAHS patients after a histopathological diagnosis of lymphoma. HPSs were controllable and remissions were achieved except for those patients with LAHS, fulminant Epstein-Barr virus-associated HPS, and an immunosuppressive state. Twenty-one (81%) of the LAHS patients had uncontrollable HPS and died of multiple organ failure and disseminated intravascular coagulation. The median survival time of LAHS patients was 83 days. In contrast, 3 (12%) of the other HPS patients died of multiple organ failure within 44 days.The clinical manifestations and the laboratory findings of LAHS and the other HPSs were too variable to establish the prognosis based only on the findings at the onset of HPS. The prognostic factors of adult HPS were found to be the underlying diseases, notably malignant lymphoma and infections, accompanied by the immunosuppressive state.
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PMID:A clinical analysis of 52 adult patients with hemophagocytic syndrome: the prognostic significance of the underlying diseases. 1159 24

An hemorheological study on whole blood filterability (WBF) was done in eleven patients bearing of sudden deafness, in a continuous way, even before clinical onset. This evaluation is making usually in animal models, but not in humans. Independently of clinical diagnosis of each patient (two cases of diabetes mellitus, one of ulcerative colitis, systemic lupus erythematosus, systemic hypertension, after blood transfusion, sepsis with disseminated intravascular coagulation, upper respiratory ways infection, after surgery, and two healthy individuals), all of them showed a decreased WBF when hearing loss appeared (from 19.97 +/- 1.15 microliters/sec to 16.87 +/- 1.21 microliters/sec). This value normalized at six or seven days from the onset in cases with some kind of hearing recovery (18.83 +/- 1.01 microliters/sec, n = 4), but did not in those with no improvement even at thirty days (17.39 +/- 0.77 microliters/sec, n = 7). There were differences in WBF values of patients with and without hearing recovery in determinations at seven and thirty days from onset. Decrease in WBF accompanies this hearing disorder and confirms the cochlear microcirculation susceptibility to the impairment of blood viscoelastic properties.
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PMID:[Non-interventional study on blood filterability changes in the clinical onset of sensorineural sudden deafness]. 1169 47

A 42-year-old woman was diagnosed as systemic lupus erythematosus (SLE), because of the findings of polyarthritis, leukopenia, positive antinuclear antibody, and positive anti DNA antibody. She was treated with predonisolone (PSL) at 10 mg per day. She was admitted to our hospital on October 2000 because of spiking high fever, skin eruption, and lymph node swelling. Since her illness of SLE was considered to be worsening, high dose of corticosteroids were given. However, high fever persisted and liver dysfunction was developed with increased serum ferritin. Her bone marrow smear showed hemophagocytosis. We made a diagnosis of hemophagocytic syndrome (HPS) complicated by disseminated intravascular coagulation (DIC). HPS was thought to be induced by viral infection, even though causative viral infection was not detected. Her general condition worsened with persistent high fever and liver dysfunction. Plasma exchange was carried for two consecutive days, followed by cyclosporine A and lipo-dexamethasone, which improved her fever rapidly. Her general condition gradually improved. Serum levels of ferritin, soluble interleukin 2 receptor (sIL 2-R), interferon-gamma and interleukin 6 decreased associated with improvements of her clinical condition. We thought plasma exchange could be effective to decrease serum levels of cytokine, which was suggested to be the pathogenic to HPS. However serum levels of IFN-gamma and IL 6 after plasma exchange did not change in this case. Further studies are required to confirm the effects of plasma exchange for HPS.
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PMID:[Case report of systemic lupus erythematosus patient with hemophagocytic syndrome, treated with plasma exchange, with specific reference to clinical profile and serum cytokine levels]. 1183 Oct 15

A case of systemic lupus erythematosus (SLE) associated with serositis presenting with disseminated intravascular coagulation (DIC) is reported. A 53-year-old woman was admitted because of a fever. Laboratory tests revealed increased plasma levels of fibrinogen degradation products (FDP) and FDP-D-dimer, high titers of anti-nuclear antibody, high serum levels of anti-DNA antibody, immune complexes, decreased serum complements, and persistent proteinuria. A CT scan showed massive ascites and pleural effusion, marked edema and swelling of the mesenterium. The patient's condition and immunological abnormalities improved after steroid therapy. The association of DIC and lupus serositis has never been described in the literature.
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PMID:Systemic lupus erythematosus associated with massive ascites and pleural effusion in a patient who presented with disseminated intravascular coagulation. 1186 7

We describe the case of a young woman with a known history of thrombocytopenia, who developed respiratory and circulatory failure thought to be caused by sepsis. She subsequently was shown to have high titres of anticardiolipin antibodies as well as lupus anticoagulant. Serological tests for various connective tissue diseases were negative. Multiple cerebral ischaemic lesions led to a poor outcome. This case highlights the potential difficulties in differentiating catastrophic anti-phospholipid syndrome from disseminated intravascular coagulation.
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PMID:Multi-organ failure secondary to catastrophic anti-phospholipid syndrome. 1193 48

The recurrence of widespread and diverse vascular lesions is a hallmark of systemic lupus erythematosus (SLE). Inflammatory and thrombotic mechanisms almost invariably associated with circulating antiphospholipid antibodies play a role in the pathogenesis of SLE-related vascular disease. Both mechanisms can coexist in the same patient. Vasculitis is most commonly induced by the local deposition of immune complexes. However, some SLE patients have an inflammatory complement-mediated vascular injury in the absence of immune complex deposition. We report on a fatal case of disseminated intravascular coagulation (DIC) in a young woman with active SLE. Hemorrhagic lesions due to localized intravascular coagulation (Shwartzman phenomenon) preceded disseminated intravascular coagulation accompanied by disseminated cardiac necrosis. Immune complex 'independent' and other mechanisms of vascular injury and states of hypercoagulability will be discussed.
Lupus 2002
PMID:Shwartzman phenomenon in a patient with active systemic lupus erythematosus preceding fatal disseminated intravascular coagulation. 1204 82

Transmittance waveform charts the changes in light transmittance on standard coagulation assays, such as the prothrombin time (PT) and activated partial thromboplastin time (APTT). Analysis and characterization of these data on photo-optical coagulation analysers provides additional qualitative and quantitative information to that obtained using the clotting time alone. The most thoroughly evaluated clinical application is that of the biphasic APTT waveform with disseminated intravascular coagulation (DIC). The degree of waveform abnormality correlates directly with the severity of haemostatic dysfunction and allows for both the prediction and monitoring from non-overt to overt DIC. As its performance is simple and rapid, this provides the means for targeting therapeutic intervention to an earlier stage of DIC. The recent identification that the mechanism underlying the biphasic waveform is a complex that exists in vivo between C reactive protein with very low density lipoprotein, provides potentially important insights into the molecular pathogenesis of DIC. Thus, in addition to the immediate clinical utility in diagnostic practice, it has important applications as a research tool. Preliminary experience in the application of this technology to the diagnosis and management of the haemophilias and the lupus anticoagulant syndrome has also provided evidence of the power and utility of waveform analysis in essentially simple clotting assays.
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PMID:Waveform analysis of clotting test optical profiles in the diagnosis and management of disseminated intravascular coagulation (DIC). 1245 11

Systematic evaluations of anemia, thrombocytopenia, and coagulopathy are essential to identifying and managing their causes successfully. In all cases, clinicians should evaluate RBC measurements alongside WBC and platelet counts and WBC differentials. Multiple competing factors may coexist; certain factors affect RBCs independent of those that affect WBCs or platelets. Ideally, clinicians should examine the peripheral blood smear for morphologic features of RBCs, WBCs, and platelets that provide important clues to the cause of the patient's hematologic disorder. Thrombocytopenia arises from decreased platelet production, increased platelet destruction, or dilutional or distributional causes. Drug-induced thrombocytopenias present diagnostic challenges, because many medicines can cause thrombocytopenia and critically ill patients often receive multiple medications. If they suspect type II HIT, clinicians must promptly discontinue all heparin sources, including LMWHs, without awaiting laboratory confirmation, to avoid thrombotic sequelae. Because warfarin anticoagulation induces acquired protein C deficiency, thereby exacerbating the prothrombotic state of type II HIT, warfarin should be withheld until platelet counts increase to more than 100,000/microL and type II HIT is clearly resolving. The presence of a consumptive coagulopathy in the setting of thrombocytopenia supports a diagnosis of DIC, not TTP-HUS, and is demonstrated by decreasing serum fibrinogen levels, and increasing TTs, PTs, aPTTs, and fibrin degradation products. Increasing D-dimer, levels are the most specific DIC parameter and reflect fibrinolysis of cross-linked fibrin. Elevated PTs or a PTTs can result from the absence of factors or the presence of inhibitors. Clinicians should suspect factor inhibitors when the prolonged PT or aPTT does not correct or only partially corrects following an immediate assay of a 1:1 mix of patient and normal plasma. In addition to factor inhibitors, antiphospholipid antibodies (e.g., lupus anticoagulant) can produce a prolonged aPTT that does not correct with normal plasma but is overcome by adding excess phospholipid or platelets. Paradoxically, a tendency to thrombosis, not bleeding, accompanies lupus anticoagulants and the antiphospholipid antibody syndrome. Transfusion of red blood cells, platelets, or plasma products is sometimes warranted, but clinicians must carefully weigh potential benefits against known risks. In critically ill patients, administering RBCs can enhance oxygen delivery to tissues. Among euvolemic patients who do not have ischemic heart disease, guidelines recommend a transfusion threshold of HGB levels in the range of 6.0 to 8.0 g/dL; patients who have HGB that is at least 10.0 g/dL are unlikely to benefit from blood transfusion. The use of rHuEPO to increase erythropoiesis offers an alternative to RBC transfusion, assuming normal, responsive progenitor cells and adequate iron, folate, and cobalamin stores. Future research should examine whether clinical outcomes from rHuEPO use in critically ill patients are important and cost-effective. Because platelets play an instrumental role in primary hemostasis, platelet transfusions are often important in managing patients who are bleeding or at risk of bleeding with thrombocytopenia or impaired platelet function. Platelet transfusions carry risks, and decisions to transfuse platelets must consider clinical circumstances. Most important, platelet transfusions are generally contraindicated if the underlying disorder is TTP or type II HIT, because platelet transfusion in these settings may fuel thrombosis and worsen clinical signs and symptoms. Plasma products can correct hemostasis when bleeding arises from malfunction, consumption, or underproduction of plasma coagulation proteins. Choice of plasma product for transfusion depends on clinical circumstances. FFP is the most commonly used plasma product to correct clotting factor deficiencies, particularly coagulopathies that are attributable to multiple clotting factor deficiency states as in liver disease, DIC, or warfarin anticoagulation. PCC or rFVIIa that is administered in small volumes may provide advantages over FFP when coagulopathies require quick reversal without risk of volume overload. Factor concentrates can replace specific factor deficiencies. Recombinant FVIIa bypasses inhibitors to factors VIII and IX and vWF. Use of rFVIIa in managing hemostatic abnormalities from severe liver dysfunction; extensive surgery, trauma, or bleeding; excessive warfarin anticoagulation; and certain platelet disorders requires further study to determine optimal and cost-effective dosing regimens. Recombinant activated protein C reduces mortality from severe sepsis that is associated with organ dysfunction in adults who are at high risk for death (APACHE scores of at least 25). In severe sepsis, levels of protein C decrease, as do fibrinogen and platelet levels. Because of its anticoagulant effect, however, drotrecogin alfa may induce bleeding. Guidelines for drotrecogin alfa use must take into account bleeding risks.
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PMID:Critical issues in hematology: anemia, thrombocytopenia, coagulopathy, and blood product transfusions in critically ill patients. 1471 Jun 93

A 31-year-old man who underwent chemotherapy and bone marrow transplantation to treat acute myeloblastic leukemia was admitted to our department complaining of high fever and hypotension. His physical examination revealed warm shock state, eruptions resembling that seen in systemic lupus erythematosus on his face and cyanosis in his fingers. We diagnosed septic shock and idiopathic skin eruption on his face. Following treatment with blood transfusion, anticoagulant, antibiotics, respirator and continuous arteriovenous hemofiltration and dialysis, the patient's condition gradually improved. The eruptions on his face first observed at admission progressed with a worsening of his disseminated intravascular coagulation (DIC), and subsided with an improvement in his DIC. A biopsy of the eruption was taken and pathological findings of the eruption revealed multiple micro-fibrin depositions of the dermis. The skin necrosis in purpura fulminans often begins in the distal extremities. But our patient developed this uncommon skin eruption on his face. Patients with an idiopathic skin eruption resembling a butterfly rash in a septic patient should be considered to complicate DIC as in the present case.
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PMID:An idiopathic skin eruption resembling a butterfly rash in a septic patient with disseminated intravascular coagulation following bone marrow transplantation. 1506 Apr 23


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