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Query: UMLS:C0409974 (lupus)
 22,386 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

We describe an 18 yr old female with systemic lupus erythematosus presenting with Evans' syndrome (autoimmune hemolytic anemia and immune thrombocytopenia) and dense myelofibrosis. Her clinical course and response to treatment were monitored with regular blood counts, serial measurements of serum procollagen I and III and periodic bone marrow examinations. This report brings to 9 the number of well-documented cases of systemic lupus erythematosis and myelofibrosis in the literature; we discuss the pathogenesis and management of patients with this apparently rare disorder, with particular reference to the role of serum procollagen measurements as markers of myelofibrosis.
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PMID:Evans' syndrome, myelofibrosis and systemic lupus erythematosus: role of procollagens in myelofibrosis. 853 92

New Zealand Black (NZB) mice spontaneously develop autoimmune disease, usually characterized by an autoimmune hemolytic anemia, and NZB genes are essential for a severe systemic lupus-like disease in (NZB x NZW)F1 mice. We have found that resting B cells from NZB mice demonstrate a pronounced defect, compared with five normal strains, in apoptosis induction after cross-linking with anti-IgM Abs. In contrast, spontaneous apoptosis of NZB B cells in culture was similar to normal strains. B cells from young (NZB x SM/J)F1 and (NZB x NZW)F1 mice underwent apoptosis normally, indicating that the NZB defect in apoptosis is a recessive trait. However, older (8-32 wk) predisease (NZB x NZW)F1 mice manifested a similar defect in apoptosis induction. The analysis of NXSM recombinant inbred mice derived from NZB and SM/J, in addition to backcross mice, suggested that the NZB apoptosis defect is a multigenic trait. Interestingly, resting B cells form B6.lpr and B6gld mice underwent apoptosis following anti-IgM treatment at a level similar to that of the C57BL/6 parental strain. Thus, the induced apoptosis of resting B cells and the NZB defect are likely not related to either Fas or Fas ligand. We propose that this phenotypic defect in apoptosis induction, or the biochemical alteration that underlies the defect, may be casually related to autoimmune disease in NZB mice and its contribution to lupus-like disease in (NZB x NZW)F1 mice.
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PMID:Resting B cells from New Zealand Black mice demonstrate a defect in apoptosis induction following surface IgM ligation. 866 26

There is a paradoxical relationship between immunodeficiency diseases and autoimmunity. While not all individuals with immunodeficiency develop autoimmunity, nor are all individuals with autoimmunity immunodeficient, defects within certain components of the immune system carry a high risk for the development of autoimmune disease. Inherited deficiencies of the complement system have a high incidence of systemic lupus erythematosus (SLE), glomerulonephritis, and vasculitis. Carrier mothers of children with chronic granulomatous disease, an X-linked defect of phagocytosis, often develop discoid lupus. Several antibody deficiencies are associated with autoimmune disease. Autoimmune cytopenias are commonly observed in individuals with selective IgA deficiency and common variable immune deficiency. Polyarticular arthritis can be seen in children with X-linked agammaglobulinemia. Combined cellular and antibody deficiencies, such as Wiskott-Aldrich syndrome, carry an increased risk for juvenile rheumatoid arthritis and autoimmune hemolytic anemia. Several hypothetical mechanisms have been proposed to explain the associations between autoimmunity and immunodeficiency. Immunologic defects may result in a failure to exclude microbial antigens, resulting in chronic immunologic activation and autoimmune symptoms. There may be shared genetic factors, such as common HLA alleles, which predispose an individual to both autoimmunity and immunodeficiency. Defects within one component of the immune system may alter the way a pathogen induces an immune response and lead to an inflammatory response directed at self-antigens. An understanding of the immunologic defects that contribute to the development of autoimmunity will provide an insight into the pathogenesis of the autoimmune process.
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PMID:The association between immunodeficiency and the development of autoimmune disease. 893 26

Double filtration plasmapheresis, one kind of fractionation plasmapheresis, was developed from membrane type plasmapheresis to remove only the pathogen and return the normal protein back to the patient. We started our automated double filtration plasmapheresis since December 1993. There were 13 patients who received one hundred treatments totally during one year period. And they are myasthenia gravis (8 patients); acute inflammatory demyelinating polyneuropathy (1 patient), multiple myeloma (1 patient); acquired factor VIII inhibitor (1 patient); autoimmune hemolytic anemia (1 patient); systemic lupus erythematous (1 patient). Technically double filtration plasmapheresis is easy to perform and time-saving. It also makes necessity of replacement fluid less frequent. Incidence of complication is rare, and this includes hypotension 2%, palpitation 1%, headache 1%, hemolysis 4%, air emboli 1%, high secondary pressure 2%, and no motality during our treatment. Clinical response is documented in cases of myasthenia gravis; acute inflammatory demyelinating polyneuropathy and acquired factor VIII inhibitor in our study. In conclusion, double filtration plasmapheresis is a time-saving, convenient, and safe therapeutic modality with rare complication. Because its effectiveness on limited kinds of diseases and costs relatively high price, thus plasmapheresis should be used in selected cases and treat aggressively if indicated.
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PMID:[Clinical experience of automated double filtration plasmapheresis]. 904 60

A 36-year-old woman with RAEB-t and severe bone marrow fibrosis undergoing autologous BMT, developed a histologically documented GVHD-like skin rash. Thereafter, autoimmune thyroiditis, autoimmune thrombocytopenic purpura and autoimmune hemolytic anemia and a lupus anti-coagulant (LAC) were diagnosed. The patient is still alive, symptom-free and in first complete remission (CR); however, all of the autoantibodies are still detectable, with the exception being the anti-erythrocyte antibody. The most outstanding feature of the present case is the polymorphism of the autoimmune events, in the absence of a coexisting systemic autoimmune disease. This patient has achieved long-term disease-free survival (DFS) in first CR despite high-risk MDS and the repeated immunosuppressant therapy required because of the complications described above; a GVL reaction somewhat similar to the autoimmune events may have contributed towards maintaining disease control.
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PMID:Multiple autoimmune events after autologous bone marrow transplantation. 915 54

The clinical features of patients with splenic marginal zone cell lymphoma (SMZCL) have rarely been reported. In the present study, immunological abnormalities, particularly hematological abnormalities, observed in SMZCL were described. Autoimmune hemolytic anemia, immune thrombocytopenia, and appearance of lupus anticoagulant were observed in 2 of 3 patients with SMZCL. Other abnormal data including monoclonal gammopathy and cold agglutinin were also observed in 2 of the 3 patients. Immunological abnormalities may be characteristic complications in patients with SMZCL and must be followed carefully, since they may be a reliable marker of this type of lymphoma activity.
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PMID:Immunological abnormalities in splenic marginal zone cell lymphoma. 937 30

To gain a better understanding of systemic lupus erythematosus (SLE) in Puerto Ricans we studied the clinical and serologic manifestations in a cohort of 134 patients. The female to male ratio was 18:1. Mean age at diagnosis was 32 +/- 12 y. The mean duration of disease and follow-up were 7.4 +/- 6.0 and 5.8 +/- 6.0 years respectively. Mortality was 3%. Photosensitivity (76.9%) and malar rash (71.9%) were the most common clinical manifestations. Arthritis was observed in 67.5% of patients. Anemia was seen in 67.2% of patients, but only 12.7% had autoimmune hemolytic anemia. Leukopenia (41.8%) and lymphopenia (64.9%) were also common. Serositis was observed in only 28%. Severe kidney damage such as nephrotic syndrome (14.2%) or renal failure (4%) was infrequent. Cardiovascular (12.7%) and neurologic (9.0%) manifestations were also uncommon. Antinuclear antibodies (ANA) were detected in 93.3%, anti-dsDNA antibodies in 54.5%, anti-Ro antibodies in 30.1% and anti-La antibodies in 14.2%. Low C3 and low C4 were observed in 38.3% and 35.7% respectively. This study suggests that Puerto Ricans with SLE present a mild form of disease predominantly manifested by cutaneous, musculoskeletal and hematologic involvement, but low prevalence of major organ damage and low mortality.
Lupus 1999
PMID:Clinical and immunological manifestations in 134 Puerto Rican patients with systemic lupus erythematosus. 1041 6

Lymphoproliferative diseases are the most common disorders associated with autoimmune disturbances. We determined the autoimmune phenotype of 64 non Hodgkin's lymphoma patients' and compared their clinicopathologic properties. Serum direct antiglobulin test [(DAT) n=64], indirect antiglobulin test [(IAT) n=61], platelet autoantibodies [(PAA) n=51], anti nuclear antibodies [n=33], anti-native DNA [n=29], anti phospholipid antibodies [n=40] and, lupus anticoagulant [n=33] were used as autoimmune markers. Twenty five patients (39%) displayed one or more autoimmune marker positivity (+). Three patients with (+) DAT and IAT had autoimmune hemolytic anemia and two patients with PAA had autoimmune thrombocytopenia. Male patients were more susceptible to autoimmunity in low grade lymphomas and the statistical difference was significant (p=0.035). Most of the autoimmune markers (+) patients had low grade and disseminated disease but this was not significant. Remission rates were not found to be different between autoimmune marker (+) and (-) patients. Although statistically not significant. median survival was longer in autoimmune marker (-) patients than in the others (50 versus 39 months). The significance of autoimmunity in NHL in a larger series of patients should be investigated in future studies.
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PMID:The significance of autoantibodies in non-Hodgkin's lymphoma. 1142 12

In order to investigate the clinical characteristics of hematological abnormality in patients with systemic lupus erythematosus (SLE) and inquire into the basis for differential diagnosis, the hematological data of 92 cases with lupus erythematosus-related hematological disorder (SLERHD) were retrospectively analyzed by use of SPSS/PC software. The results showed that these patients were short of specificity in clinical manifestation and hemogram, however, all cases possessed multiple SLE-related autoantibodies, increase of serum globulin level and varying extent dermal and arthral signs. The incidence of primary or initial symptom in the 92 cases was as follow: 65 anemia (72.8%), 39 purpura (42.4%), 17 hemolytic anemia (18.5%), 56 leukopenia (60.9%), 54 thrombocytopenia (58.7%), and 41 pancytopenia (44.6%). The bone marrow examinations showed that the cellularity of nucleated cells was mostly normal, and active proliferation in 57 cases (61.9%) and hypercellularity in 35 cases (38.1%); the G/E ratio was normal in majority, and G/E ratio > 3 in 59 cases (64.1%) and < 3 in 33 cases (35.9%) and G/E < 1 in 17 cases with hemolytic anemia Coombs' test positive; megakaryocyte counts were normal in 11 cases (11.9%), increase in 80 cases (86.9%) and lower than 7/marrow smear in 1 case (1.1%). Neutrophil alkaline phosphatase staining was negative in all of the cases. From above data it is concluded that patients with SLERHD are varied in clinical and blood pictures, but all patients are provided with multiple SLE-related autoantibodies, globulinemia and dermal and arthral signs. It is easy to identify SLERHD from aplastic anemia, myelodysplastic syndrome, idiopathic thrombocytopenic purpura, autoimmune hemolytic anemia and Evans' syndrome by comprehensive and detailed clinical and laboratory examinations.
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PMID:[Clinical features of hematological abnormality in systemic lupus erythematosus-related hematological disorders]. 1251 74

Hyperinfection in strongyloidiasis has been associated with corticosteroid treatment. Other immunodepressive conditions also seem to facilitate the state of hyperinfection. The etiologic diagnosis of this parasitosis can be difficult to reach and a positive urine microscopy is unusual. We report two patients under corticosteroid therapy with disseminated strongyloidiasis; both had eosinophilia. The first patient, followed for 8 years for autoimmune hemolytic anemia, recently developed abdominal symptoms. A colonoscopy was performed 1 month before admission and the biopsy was thought to show nonspecific changes. At admission, few larvae of Strongyloides stercoralis were disclosed by urine microscopy, and a review of the colonic biopsy uncovered a few larvae of Strongyloides. The patient received anti-helmintic therapy with a dramatic improvement. The second patient, under treatment for lupus erythematosus for 3 years, was admitted with pulmonary symptoms and during admission developed massive gastrointestinal bleeding. Disseminated strongyloidiasis was discovered only at autopsy. The low suspicion index for strongyloidiasis resulted in delaying the etiologic diagnosis in one patient and in failing to diagnose the disease in the other. The morphologic features of the parasite in the two cases are presented with emphasis on the difficulties of recognizing the larvae in the intestinal biopsy.
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PMID:Hyperinfection syndrome in strongyloidiasis: report of two cases. 1271 33


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