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)

Acutely, hemin sensitizes endothelial cells to oxidants but chronically protects the endothelium through the induction of ferritin. By releasing its heme, methemoglobin can sensitize endothelial cells in a fashion similar to free hemin. Furthermore, prolonged incubation with the endothelium allows methemoglobin to induce heme oxygenase and ferritin and concomitantly to modulate oxidant-mediated cytotoxicity. Methemoglobin but not hemoglobin, metmyoglobin or cytochrome c induces heme oxygenase and ferritin. Heme needs to be released from methemoglobin, since sodium cyanide, haptoglobin, and hemopexin inhibit the induction of these proteins. Neutrophils can oxidize hemoglobin to methemoglobin, which can subsequently induce both heme oxygenase and ferritin. We speculate that in shock with disseminated intravascular coagulation, marginated PMNs oxidize hemoglobin to heme-releasing methemoglobin. If critical defenses such as haptoglobin and hemopexin are overwhelmed, heme enters the endothelin cells, sensitizing them to oxidant damage. Endothelial cell adaptation via heme-induced heme oxygenase and ferritin production might limit ultimate progression to pulmonary and other vascular leak syndromes.
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PMID:Endothelial cell heme oxygenase and ferritin induction by heme proteins: a possible mechanism limiting shock damage. 130 86

Disseminated intravascular coagulation (DIC) was induced in rabbits by administration of antibody and antigen. The rabbits were sensitized passively by i.v. injection of antiferritin antiserum and challenged simultaneously by an i.p. inoculation of ferritin. After the challenge, circulating white blood cells, platelets and plasma fibrinogen levels showed an early fall, reaching minimum values at 3, 10 and 6 h, respectively. Fibrin thrombi appeared first at 2 h, reached a maximum at 5-7 h, and had mostly disappeared by 24 h. Formation of fibrin thrombi was frequent in the lung, liver, kidney and spleen. Early morphological changes included neutrophilic infiltration and accumulation of platelets in capillaries. Ferritin-antiferritin complexes were noted among fibrin thrombi or phagocytized by reticuloendothelial cells and neutrophils. The capacity of Kupffer cells to remove circulating immune complexes was saturated transiently; at this time fibrin thrombosis in various organs was most widespread and severe. It seems likely that formation of antigen-antibody complexes in the microcirculation initiates activation of platelets and neutrophils with subsequent release of mediators responsible for triggering DIC. Activation of complement was another possible factor inducing the reaction. In addition, blockade of the reticuloendothelial system promotes the progression of DIC. It is considered that the methods described constitute a useful model for further elucidation of immune complex-induced DIC.
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PMID:Immune complex-induced disseminated intravascular coagulation (DIC). An experimental study. 222 Mar 95

A 57-year-old man was admitted to our hospital with high fever and nasal obstruction. The diagnosis of T cell type malignant lymphoma (T-ML) was made by the biopsy of left nasal cavity tumor. After admission, his general condition was improved by chemotherapy and radiotherapy, but relapsed a month later. He was then treated with chemotherapy, and the partial remission was obtained. During the clinical course, he had a high fever again without any significant infections or exacerbation of T-ML. The data of coagulation system showed DIC. The levels of serum ferritin and LDH were extremely elevated. Bone marrow aspiration showed markedly increased hemophagocytic histiocytes. These data suggested that he was complicated by DIC and hyperferritinemia closely associated with hemophagocytic histiocytosis a part from the underlying T-ML. Causes of DIC and hyperferritinemia associated with hemophagocytic histiocytosis in the present case were discussed.
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PMID:[T-cell malignant lymphoma with hemophagocytic histiocytosis, hyperferritinemia and disseminated intravascular coagulation syndrome]. 228 69

Serum ferritin level was measured serially in three malignant histiocytosis (MH) and five virus-associated hemophagocytic syndrome (VAHS) patients. Serum ferritin levels were greater than 1000 ng/ml at the early phase of disease. When disseminated intravascular coagulation (DIC) developed in patients, serum ferritin levels elevated further to greater than 12,000 ng/ml. MH patients were treated by combination chemotherapy, and VAHS patients were given prednisone. Each MH and VAHS patient died within 1 week from the onset of DIC, without decrease of serum ferritin level. One MH patient with continued high serum ferritin levels for 3 months after DIC also died. The remaining patients with decreased serum ferritin values less than 1000 ng/ml at 3 months after DIC are now alive with normal serum ferritin levels. These results suggest that serum ferritin level in histiocytic proliferative disorders is a useful indicator of disease activity in both neoplastic and reactive conditions rather than only a marker of malignant process.
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PMID:High serum ferritin level as a marker of malignant histiocytosis and virus-associated hemophagocytic syndrome. 335 4

Hemostatic function of 129 patients with cancer of the digestive system was studied on the clinical point of view. Activator (A) and inhibitor (I) of fibrinolysis of 94 cancer tissues were determined by Malone's method. The following results were obtained: Latent DIC state was observed in the patients with advanced stage. Great majority of the patients with PT less than or equal to 85%, antithrombin III (AT III) less than or equal to 25 mg/dl, FDP greater than or equal to 5 micrograms/ml, alpha 1 antitrypsin (alpha 1 AT) greater than or equal to 340 mg/dl, plasminogen (plg) less than or equal to 10 mg/dl and alpha 2 plasmin inhibitor (alpha 2 PI) less than or equal to 80%, were eligible only for non-curative operation on the preoperative evaluations. Persistent decreases in PT, AT III, plg and alpha 2 PI mean poor prognosis, which were seen within about 6 months prior to death. In gastric cancer patients, these abnormalities showed correlations with serum choline-esterase, albumin and ferritin, and post-operative changes of these parameters suggested the recurrence. There were I activities in the cancer tissues which were scarcely detected in the normal tissues. Some differences in A/I ratios were observed on types of organs involved, histological types and differentiative degrees. There were no correlations between the hemostatic state and A/I ratios. These results indicate the clinical usefulness of the hemostatic functions of the cancer patients and the fibrinolytic properties of the cancer tissues, and also suggested that tumor bearing state, liver function and non-specific stimulating mechanisms participate in the appearance of the abnormalities.
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PMID:[Hemostatic abnormalities of the patients with cancer. Clinical significance and fibrinolytic properties of the cancer tissues]. 620 15

A 71 year-old man with adult onset Still's disease was admitted to our hospital because of fever, sore throat, myalgia and macular nonpruritic salmon pink eruption. He was treated with prednisolone, 40 mg daily and these symptoms disappeared. When the dose of prednisolone was reduced to 30 mg daily, he began to notice fever. 5 days later he developed adult respiratory distress syndrome (ARDS). The dose of prednisolone was increased to 50 mg daily and oxygen administration was started. All symptoms began to improve immediately and the dose of prednisolone was decreased to 40 mg daily. 10 days later he noticed fever and skin rash. Laboratory investigation showed platelet counts of 69,000/mm3, a ferritin of 37,000 ng/ml, and increased fibrinogen degradation product, indicating increased activity of adult onset Still's disease associated with disseminated intravascular coagulation (DIC). The dose of prednisolone was again increased to 60 mg daily, and 100 mg of nafamostat mesilate was administrated intravenously. All above symptoms associated with adult onset Still's disease and DIC disappeared. The dose of prednisolone was gradually decreased and the clinical course was uneventful with daily administration of 10 mg of prednisolone. Although there are a couple of case report which described the association of adult onset Still's disease with either ARDS or DIC, the association of adult onset Still's disease with both ARDS and DIC have not been reported yet.
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PMID:[A case of adult onset Still's disease complicated with adult respiratory distress syndrome and disseminated intravascular coagulation]. 755 55

Hemophagocytic syndrome (HPS) presents with fever, pancytopenia, liver dysfunction and increase in hemophagocytic histiocytes in various organs. Although there are two major classifications of HPS in adults, malignant and reactive histiocytosis, it is often very difficult to distinguish between these disorders. We analyzed the laboratory data of patients with HPS to evaluate prognostic factors. Of 34 patients, 14 survived, and 20 died. The median age of survivors was 29.6+/-11.5 yr significantly younger than those who died (54.7+/-17.8 yr). Twenty patients had no obvious underlying disease, the other 13 had hematological malignancies or viral infections. Comparison of laboratory data revealed that nonsurvivors had significantly lower Hb and platelet values on admission. During treatment, worsening of anemia and thrombocytopenia, increase of transaminase and biliary enzymes were similarly more prominent. Risk factors associated with death were: age over 30 yr, presence of disseminated intravascular coagulation, increased ferritin and beta2-microglobulin, anemia accompanied by thrombocytopenia and jaundice. Our data suggests that patients with HPS and any of these risk factors should be treated aggressively with sufficient chemotherapy and supportive care.
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PMID:Prognostic factors of hemophagocytic syndrome in adults: analysis of 34 cases. 933 23

A potentially fatal hemophagocytic syndrome (HPS) has been noted in patients with reactive HPS. We describe 2 patients with reactive HPS treated with a regimen of therapeutic plasmapheresis and evaluate the efficacy of plasmapheresis for fatal HPS. Case 1 was a 31 year-old woman who had been treated for systemic lupus erythematosus (SLE) with corticosteroid hormones and immunosuppressants. She presented with persistent leukopenia and thrombocytopenia with spiking fever. She had an elevated level of serum ferritin, liver dysfunction, coagulopathy, and plasma inflammatory cytokines. Her bone marrow smear disclosed numerous hemophagocytosis of histiocytes. She was administered therapeutic plasmapheresis with total plasma exchange by fresh frozen plasma. There was an immediate and prominent decrease of cytokines, and she completely recovered. Case 2 was a 34 year-old woman who had been receiving high doses of corticosteroids and plasmapheresis for severe Stevens-Johnson's syndrome. After 18 months, she presented with physical and laboratory findings resembling lupus-like conditions and was administered high doses of corticosteroids and immunosuppressants. Human parvovirus B19 infection was detected by IgM and IgG antibodies and viral DNA from a bone marrow sample; moreover, a bone marrow smear disclosed findings of HPS. Repeated therapeutic plasmapheresis was effective for improving her symptoms and laboratory abnormalities; however, she suffered from septic methicilline resistant staphylococcus aureus infection and finally died of a brain hemorrhage resulting from disseminated intravascular coagulation (DIC).
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PMID:The efficacy of therapeutic plasmapheresis for the treatment of fatal hemophagocytic syndrome: two case reports. 1022 60

Adult-onset Still's disease, the adult variant of the systemic form of juvenile arthritis, is an uncommon systemic inflammatory disorder of unknown etiology characterized by high spiking fevers, neutrophilic leukocytosis, arthritis, and an evanescent rash. There is often a delay in reaching a firm diagnosis. Differential diagnoses include infection, malignancy, and various immunologic disorders. Increased ferritin levels are of particular value in establishing the diagnosis. Clinical response to high-dose corticosteroids may be dramatic. We report a case of a 29-year-old woman who had recently been investigated for fever of unknown origin, and who presented to our hospital with high fever and hypotension. Her condition rapidly deteriorated with the development of ARDS, disseminated intravascular coagulation, and shock. The patient had a markedly elevated serum ferritin concentration of 26,000 ng/mL. High-dose pulse methylprednisolone therapy resulted in a remarkable clinical improvement. Such a severe case of systemic inflammatory response syndrome, masquerading as septic shock, has not been reported previously.
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PMID:Severe systemic inflammatory response syndrome with shock and ARDS resulting from Still's disease: clinical response with high-dose pulse methylprednisolone therapy. 1037 76

A 32-year-old woman in the 16th week of pregnancy was admitted to our hospital because of high fever. Laboratory findings disclosed pancytopenia and extremely elevated serum LDH and ferritin levels. Coagulation tests showed disseminated intravascular coagulation. Serum soluble interleukin-2 receptor, tumor necrosis factor-alpha, and interleukin-6 levels were high, but serum interferon-gamma was below the detectable limit. Reactive Epstein-Barr virus (EBV) infection was diagnosed on the basis of a high titer of IgG antibodies to the EBV capsid antigen and early antigen. EBV was demonstrated in the peripheral blood and bone marrow cells by polymerase chain reaction. Mature histiocytosis and hemophagocytosis were detected in the bone marrow. A diagnosis of EBV-associated hemophagocytic syndrome (EBV-AHS) was made. Neither prednisolone (PSL 30 mg/day, P.O.) nor methylprednisolone (m-PSL) pulse therapy (1,000 mg/day for 3 days) induced a response. Thereafter, treatment with m-PSL pulse therapy (1,000 mg/day for 3 days) and i.v. administrations of high-dose immunoglobulin (20 g/day for 3 days) in combination with acyclovir (750 mg/day) and gabexate mesilate (2 g/day) induced remission of the disease. Maintenance therapy consisted of PSL (5 mg/day, P.O.) and camostat mesilate (600 mg/day, P.O.). The patient delivered a healthy male infant in the 35th week of pregnancy via natural birth. Reports of pregnant women with EBV-AHS are rare, and the choice of therapy has not yet been established. The present case study suggested the above combination treatment is useful and safe, and capable of changing the fulminant course of EBV-AHS during pregnancy without the use of anticancer drugs.
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PMID:[Epstein-Barr virus-associated hemophagocytic syndrome during mid-term pregnancy successfully treated with combined methylprednisolone and intravenous immunoglobulin]. 1065 79


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