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)

Platelets play a central role in haemostasis. Not only are they involved in aggregatory and agglutination responses but they are also implicated in the clotting system. The conversion of prothrombin to thrombin, in the presence of coagulation factors Va, Xa and calcium ions, is termed prothrombinase activity. For optimal expression of this process a negatively charged phospholipid surface is required. Platelets can provide such an environment, by exposing negatively charged phospholipids at their external plasma membrane, by a 'flip-flop' process whereby negatively charged phospholipids, predominantly phosphatidylserine, move from the inner plasma membrane leaflet to the outer leaflet upon the activation of platelets by certain agonists. Such agonists include collagen and thrombin and the amount of prothrombinase activity expressed is well correlated with the propensity of the agonist to activate platelet calcium-dependent protease, calpain. This enzyme is then thought to act upon platelet cytoskeletal components, thus breaking the restraining action of the cytoskeleton upon the platelet plasma membrane and facilitating 'flip-flop'. The platelet plasma membrane is therefore a dynamic surface capable of catalytic functions in coagulation systems. Recent research has high-lighted abnormalities in platelet prothrombinase expression in certain disease states. These include Bernard-Soulier syndrome, essential thrombocythaemia and conditions where the lupus anticoagulant may be present.
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PMID:Platelet prothrombinase in health and disease. 213 Sep 28

Hemapheresis has been used in a variety of clinical states, primarily for its ability to remove an offending component, likely be either plasma or cellular elements. We have reviewed 43 cases of therapeutic hemapheresis over the past two years at Veterans General Hospital. There were 20 cases of plasmapheresis, 7 cases of leukapheresis and 16 cases of thrombocytapheresis. Most of them had satisfactory and desirable effects except a few cases in therapeutic plasmapheresis. Among 20 cases of therapeutic plasmapheresis, all but 2 cases of systemic lupus erythrematosus had transient clinical improvement. Average decrease of leukocyte count in 11 procedures of leukemic patients was 37%. As for the myeloproliferative disorders with thrombocytosis, the average drop of platelet count was 41%. The side effects of therapeutic hemapheresis were not infrequent. There were 56 recorded side effects with one sudden death among 94 procedures. Hemapheresis is useful in certain clinical conditions but more judicious application should be considered.
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PMID:Clinical application of therapeutic hemapheresis. 216 55

We report the case of a 13-yr-old patient with retarded growth and a 2-yr background of asthenia, anorexia, and fever, whose laboratory data revealed anemia, thrombocytosis, an elevated erythrocyte sedimentation rate, ferropenia, hyperglobulinemia, hyperfibrinogenemia, and presence of a lupus-like circulating anticoagulant. Clinical studies revealed a tumor-like overgrowth in the gastric wall, and surgery confirmed its subserosal localization in the gastric fundus. After total removal of the mass, the systemic manifestations disappeared. The pathological study revealed the existence of the hyaline-vascular variety of Castleman's disease. Having reviewed the medical literature, we have not found a single unquestionable case of gastric Castleman's disease, although three other cases have been described as gastric pseudolymphoma which, when analyzed, could correspond to typical cases of Castleman's disease. Likewise, this is the second case associated with a circulating anticoagulant of lupoid characteristics. We conclude that Castleman's disease should be included in the differential diagnosis of gastric lesions of lymphoid nature and in the series of processes associated with lupus anticoagulant.
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PMID:Gastric Castleman's disease with a lupus-like circulating anticoagulant. 249 41

Plasma prostacyclin (PGI2) degradation rates were measured at 1, 5, 15 and 30 min in a group of patients with platelet quantitative disorders of various pathogeneses, including 13 with thrombocytosis, 16 with thrombocytopenia from impaired production in the bone marrow, 11 with thrombocytopenia from peripheral destruction, and 28 normal, healthy persons. Patients with thrombocytosis had a low PGI2 degradation rate, whereas patients with thrombocytopenia due to impaired production had a high PGI2 degradation rate. Of the patients with thrombocytopenia caused by peripheral destruction, six with idiopathic thrombocytopenia purpura (ITP) had a slow PGI2 degradation in contrast to five with systemic lupus erythematosus (SLE) - four concurrently had cryoglobulinemia - who had a rapid PGI2 degradation. The findings suggest that: (1) a platelet-derived substance in the human plasma may have a PGI2 stabilising activity; (2) presence of cryoglobulin or immune complex in plasma may interfere with PGI2 stability.
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PMID:Prostacyclin degradation in patients with quantitative platelet disorders. 329 Sep 5

Autoreactive antibodies or immune complexes may accelerate clearance of mature erythrocytes, leukocytes, and platelets from the circulation in patients with rheumatologic and immunologic disorders. The most compelling evidence for immune injury to hematopoietic cells exists in patients with systemic lupus erythematosus and patients with Felty's syndrome and its variants. These disorders may also cause tissue inflammation, which in turn commonly results in underproduction of erythrocytes and development of thrombocytosis. However, recent evidence indicates that underproduction of hematopoietic cells may also result from immune injury to cellular elements in the bone marrow. In many laboratories, sensitive techniques are now clinically available for the detection of cell-associated immunoglobulin and complement. These assays have helped confirm the role of antibody in the pathogenesis of autoimmune hemolytic anemia and idiopathic thrombocytopenic purpura. However, recent data indicate that there is probably a continuum between the amount of immunoglobulin and complement found on normal cells and that found in a variety of disease states. In several of these disorders, additional evidence will be required to establish that the increase in cell-bound immunoglobulin leads to a decrease in the life-span of the cell. In order to provide significant help to the clinician managing an individual patient, these serologic tests must be capable of identifying the portion of the cell-associated protein actually involved in the destructive process. The availability of monoclonal reagents capable of identifying restricted regions on cell-bound immunoglobulin may help identify molecules bound specifically as antibody and may help identify the antigens involved in autoimmune disorders.
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PMID:Antibodies reactive with surface membranes of cellular elements in the blood. 634 Dec 6

The several kinds of mice that spontaneously develop acute systemic lupus erythematosus (SLE)--BXSB males, MRL/l males and females, and (NZB X W)F1 females--have a 15-20% incidence of degenerative vascular disease (DVD) and myocardial infarcts (MI) in which the affected coronaries contain deposits of immunoreactants, presumably in the form of immune complexes. Among the F1 hybrid crosses of SLE mice, only the (NZW X BXSB)F1, (W X B)F1 male has a significantly higher incidence of DVD/MI (80%). Search for possible causes of this high incidence of myocardial infarcts revealed several unique features of this mouse: hypertension, thrombocytosis, and early onset of circulating immune complexes and glomerulonephritis. Our attempts to prevent this DVD/MI focused on: reduction of hypertension, prevention of thrombosis, and immunosuppression. Immunosuppression by Cytoxan resulted in almost complete prevention of both the SLE disease and DVD/MI. Administration of bretylium, an antihypertensive and anti-arrhythmic agent, resulted in reduction of blood pressure and the severities of glomerulonephritis, DVD, and MI; it also slightly reduced the levels of circulating immune complexes and leukocytosis. Of the 4 antithrombotic agents used, only aspirin showed some reduction in the incidence of DVD/MI and delay of glomerulonephritis-associated mortality.
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PMID:The role of hypertension in the vascular disease and myocardial infarcts associated with murine systemic lupus erythematosus. 663 92

High dose immunoglobulin infusions showed a marked effect on platelet counts in eight out of nine chronic ITP patients and in one SLE patient. In the comparison of different IgG-preparations, the pepsin treated IgG F (ab')2 showed no platelet elevation while the sulfonated did. The elevated platelet count could not be maintained after discontinuation of IgG infusions, but in six out of ten patients the platelet level remained above the pretreatment values. This new treatment seems to be safe and effective in adulthood ITP.
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PMID:Clinical effect of intravenous immunoglobulin on chronic idiopathic thrombocytopenic purpura. 668 78

Hematological abnormalities in scleroderma indicate a specific complication of the disease itself, or an associated illness. Among 180 patients with scleroderma, anemia was detected in 25% and was attributed to chronic inflammatory disease (usually an overlap syndrome), bleeding mucosal telangiectases as part of the CREST syndrome, intestinal malabsorption, and microangiopathic hemolysis. Leukocytosis, present in 14%, was correlated with active myopathy and/or advanced visceral involvement while leukopenia was suggestive of a crossover with systemic lupus erythematosus. Thrombocytopenia was often a manifestation of microangiopathy, and thrombocytosis was associated with an arteritis or a tumor syndrome.
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PMID:Hematological abnormalities in scleroderma. A study of 180 cases. 677 74

A case of pure red cell aplasia (PRCA) with various complications polyarthritis, angitis, acute renal failure and DIC was successfully treated with steroid pulse therapy was described. A 55-year-old woman was hospitalized with a 9-month of intermittent but progressive joint pain, morning stiffness, general fatigue, and fever. Her initial laboratory evaluation revealed a hemoglobin of 4.4 g/dl and absence of reticulocyte. Her bone marrow aspirate showed no erythroblast which was compatible with a diagnosis of PRCA. Marked leukocytosis and thrombocytosis, positive antinuclear antigen, elevation of gammaglobulin and C-reactive protein and the presence of polyarthritis and angitis which was confirmed by renal angiography, indicated an underlying autoimmune disorders. Steroid pulse therapy was administered at 500 mg/day for 3 days, resulting in the complete response in both red cell aplasia and above findings. PRCA is known to be associated with systemic lupus erythematosus and rheumatoid arthritis very rarely, but this case did not fulfill the criteria of known collagen diseases, and there is no previous report representing PRCA with various complications such as polyarthritis, angitis and acute renal failure. This case may help us to understand more about the relationship between PRCA and autoimmune disorders.
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PMID:[Pure red cell aplasia complicated with polyarthritis, angitis, and acute renal failure]. 825 11

Several classifications have been proposed for erythermalgia according to causality (primary or secondary) or age of onset (presuming that all early-onset are primary). Recently a classification in three types of erythromelalgia have been proposed. Erythromelalgia and erythermalgia are defined as two independent and completely different disease entities. Clinically there are three different types of recurrent red, warm and burning pain in the extremities. That need to be distinguished for effective treatment according to their etiology: erythromelalgia in thrombocythaemia, primary erythermalgia and secondary erythermalgia. We recently observed 19 cases of erythermalgia; nine had primary erythermalgia; in 6 of 9, symptoms were relieved with aspirin. Ten of them had a secondary erythermalgia: 5 due to myeloproliferative disorders (erythromelalgia), 2 systemic lupus erythematosus and 3 to drugs. We used a two-level classification with a first level of primary or secondary erythermalgia, and a second level for primary erythermalgia, of familial or nonfamilial primary and for secondary erythermalgia, of thrombocythaemia disorders or other.
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PMID:[Classification of erythermalgia]. 875 86


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