Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0027947 (neutropenia)
 17,527 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

The infant of a mother with systemic lupus erythematosus (SLE) developed an extensive cutaneous eruption at 5 weeks of age. Biopsy findings were consistent with cutaneous lupus erythematosus (LE). Splenomegaly, anemia, neutropenia, and depressed total hemolytic complemtnt levels were additional findings. The course was benign, and all manifestations disappeared by 4 months of age. Fifty-two previously reported infants with cutaneous lesions, congenital atrioventricular heart block, or hematologic manifestations of neonatal LE are reviewed.
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PMID:Neonatal lupus erythematosus. 51 88

A case history is presented of the occurrence of a high binding capacity for native DNA in the serum of a patient on phenylbutazone. This reverted to normal on stopping the drug. The patient also had a reversible neutropenia and leucopenia, and it is suggested that the high anti-DNA binding capacity was a feature of a drug-induced lupus-like phenomenon.
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PMID:Antinative DNA antibodies as a reaction to pyrazole drugs. 108 78

A variety of antihypertensive drugs have been introduced into clinical practice at excessively high dose. Examples include most thiazide diuretics, propranolol, oxprenolol, atenolol, methyldopa, hydralazine and captopril. These very high doses have usually resulted from studies in which doses have been increased at regular intervals until the desired antihypertensive effect has been achieved or until unacceptable adverse effects have resulted. Frequently the starting doses were too high and the intervals between dose adjustment too short. In many cases these large doses resulted in unnecessary adverse effects--the adverse biochemical effects of thiazide diuretics, nephrotic syndrome, taste disturbances and neutropenia with captopril, the lupus syndrome with hydralazine and the central nervous system effects of methyldopa. Parallel group design with single doses and sufficient statistical power to distinguish between the upper and lower ends of the antihypertensive dose-response relationship should replace the dose-escalating design.
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PMID:Dose-response relationships with antihypertensive drugs. 128 75

Deep cutaneous lesions are seldom encountered in lupus erythematosus. The disease described in the literature as lupus erythematosus profundus or lupus erythematosus panniculitis usually occurs in middle-aged women. The authors report a case of deep lupus erythematosus which was exceptional in that it occurred in an 11-year old girl. The lesions were situated on the face and consisted of solid subcutaneous nodules and clear-cut ulcerations leaving atrophic pigmented scars. The histological image of a nodule was one of lobular lymphocytic panniculitis with homogeneous hyalinization of adipose nodules, to which must be added periadnexal and perivascular dermo-epidermal lymphocytic infiltrates. Granular IgM deposits arranged along the dermo-epidermal junction were observed at direct cutaneous immunofluorescence. Laboratory examinations showed leucopenia (3,300/mm3) with neutropenia (1,100/mm3) and the presence of antinuclear antibodies at 1/100 speckled fluorescence, as well as antibodies directed against native DNA. Studies of renal function and for complement gave normal results. The other causes of lobular panniculitis were excluded. The lesions regressed within 3 weeks under hydroxychloroquine; this drug was also successful in arresting a relapse consecutive to withdrawal of treatment. The authors have analysed the 17 paediatric cases of deep lupus erythematosus and were able to determine their main characteristics: 1. The lesions occur mostly in girls (70 p. 100). They are located electively on the face and the lateral aspect of the shoulders. They consist of well-limited, solid or hard subcutaneous nodule which may congregate to form plaques. The epidermis may be normal or pathological, poikilodermic, looking like a discoid or ulcerated lupus erythematosus. The lesions regress, leaving a characteristic atrophic scar.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:[Deep lupus erythematosus in children]. 148 54

We review our experience with low-dose intravenous pulse cyclophosphamide as treatment of biopsy-proven lupus nephritis. Seventeen patients were treated with 2-4 (mostly 3) weekly low-dose intravenous pulses of cyclophosphamide (500 mg) and moderate doses of prednisolone (0.5 mg/kg/day), followed by an oral immunosuppressive drug (either azathioprine or cyclophosphamide). As compared with the classical monthly high-dose cyclophosphamide regimen, this weekly low-dose regimen induced neutropenia in one patient only. The incidence of herpes zoster was very low (6%). At the end of the follow-up period (15 +/- 8 months), two patients required chronic ambulatory peritoneal dialysis. The 14 patients that could be evaluated improved their mean serum albumin from 30 +/- 7 to 37.5 +/- 7 g/l (mean +/- SD; P < 0.01) and their mean serum creatinine fell from 125 +/- 119 to 101 +/- 66 mumol/l (not significant). Mean DNA binding dropped from 71 +/- 29 to 26 +/- 27% (P < 0.001) and mean complement fraction C4 levels increased from 14 +/- 8 to 28 +/- 18 mg/dl (P < 0.05). The mean daily prednisolone dose was dramatically reduced from 26 +/- 8 to 10 +/- 4 mg (P < 0.001). Although this preliminary and retrospective study clearly needs validation with a larger cohort followed for a longer period, it seems that a treatment combining moderate doses of steroids and 3-4 weekly low-dose intravenous pulses of cyclophosphamide, followed by oral immunosuppression, is well tolerated and beneficial--at least in the short term--for most patients with severe lupus nephritis.
Lupus 1991 Nov
PMID:Short course of weekly low-dose intravenous pulse cyclophosphamide in the treatment of lupus nephritis: a preliminary study. 184 61

A prospective study of IgG and IgM isotypes of anticardiolipin antibodies (aCL) in a series of 100 patients with systemic lupus erythematosus was carried out. To determine the normal range of both isotype titres a group of 100 normal control serum samples was studied and a log-normal distribution of IgG and IgM isotypes was found. The IgG anticardiolipin antibody serum was regarded as positive if a binding index greater than 2.85 (SD 3.77) was detected and a binding index greater than 4.07 (3.90) was defined as positive for IgM anticardiolipin antibody. Twenty four patients were positive for IgG aCL, 20 for IgM aCL, and 36 for IgG or IgM aCL, or both. IgG aCL were found to have a significant association with thrombosis and thrombocytopenia, and IgM aCL with haemolytic anaemia and neutropenia. Specificity and predictive value for these clinical manifestations increased at moderate and high anticardiolipin antibody titres. In addition, a significant association was found between aCL and the presence of lupus anticoagulant. Identification of these differences in the anticardiolipin antibody isotype associations may improve the clinical usefulness of these tests, and this study confirms the good specificity and predictive value of the anticardiolipin antibody titre for these clinical manifestations.
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PMID:Isotype distribution of anticardiolipin antibodies in systemic lupus erythematosus: prospective analysis of a series of 100 patients. 210 99

Hematologic abnormalities are common in association with collagen diseases, specially Systemic Lupus Erythematosus and include anemia, neutropenia, thrombocytopenia with alterations in lymphocyte subpopulations. On the other hand, patients with unexplained fibrosis of the bone marrow (the syndrome of idiopathic myelofibrosis or primary myelofibrosis) have clinical and laboratory evidence of immunologic dysfunction. Clinical findings include the presence of arthritis, vasculitis and erythema nodosum. Laboratory abnormalities include the presence of circulating immune complexes, antinuclear antibodies, positive direct Coombs test, elevated latex fixation and a circulating lupus type anticoagulant. Total hemolytic complement markedly depressed has also been reported. These data suggest that immunologic mechanisms associated with activation of the complement system play an important role in the disease process of some patients with agnogenic myeloid metaplasia with myelofibrosis. A review of the literature revealed that myelofibrosis occurring in the setting of collagen diseases is rare. However, a role for immunologic factors in the pathogenesis of myelofibrosis is also supported by the patients with coincident well defined collagen disease and myelofibrosis. In this report, we present two patients with such an association. Case 1 was a 58-year-old male with a two year duration history of rheumatic arthritis. He had bone erosions on hands, splenomegaly and myelofibrosis. Rheumatoid factor (latex) was positive: 1:2560. He had positive LE cells and hypocomplementemia: 37 CH50/ml (NV 70-150). The patient did not meet criteria for SLE. Case 2 was a 36-year-old female admitted because of dyspnea and fever. Diagnosis of myeloid metaplasia with myelofibrosis and progressive systemic sclerosis had been made four years before hand.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:[Coexistence of myelofibrosis and collagen diseases]. 213 Feb 12

High dose intravenous gammaglobulin (0.4 g/kg/day) for five days, causes a rapid rise in the platelet count in immune thrombocytopenic purpura (ITP). The response in chronic ITP is transient making it useful in emergency situations. Efficacy in immune mediated neutropenia and warm antibody haemolytic anaemia has been reported but in limited numbers. We have used this therapy in twenty-three adults with ITP, one patient with immune neutropenia, four patients with warm antibody autoimmune haemolytic anaemia and one case of thrombotic thrombocytopenic purpura (TTP). In ITP only four of the twenty patients failed to respond to gammaglobulin and of these, three had a positive antinuclear factor but no other signs of systemic lupus erythematosis. The sole patient with neutropenia treated with IgG failed to show any response. In three patients with haemolytic anaemia parameters of haemolysis improved during therapy. The patient with TTP suffered a cerebrovascular accident during therapy prior to a documented response. High dose intravenous immunoglobulin rapidly elevates the platelet count in acute and chronic ITP. Its use in other immune mediated haematological conditions has yet to be fully evaluated.
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PMID:High dose intravenous gammaglobulin in immune haematological disease. 244 Jul 41

The systemic rheumatic diseases are commonly complicated by hematologic abnormalities. Five frequently encountered and clinically relevant complications are reviewed. They include the anemia of chronic disease, neutropenia, autoimmune thrombocytopenic purpura, the lupus inhibitor, and hematologic malignancies. The pathophysiology and treatment of each of these conditions are discussed in this review.
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PMID:Hematologic complications of rheumatic disease. 311 57

Sixteen pediatric patients diagnosed with a variety of autoimmune-mediated hematocytopenias were treated with one to 50 courses of intravenous gamma globulin (IVIG), pH 4.25, over the course of one to 30 months. Thirteen patients had immune thrombocytopenic purpura (ITP), two had autoimmune neutropenia, and one had autoimmune hemolytic anemia. In one patient, chronic ITP was associated with systemic lupus erythematosis, and in a second patient, acute ITP was the presenting manifestation of infection with human immunodeficiency virus. Initial therapy consisted of 400 mg/kg/dose daily for five days for the first seven patients treated, and 1,000 mg/kg/dose daily for two days for the remaining nine patients. In 15 of 16 patients, there was a response to IVIG therapy. In nine of 16 patients, maintenance IVIG therapy for two to more than 30 months was required. Minimal toxicity was experienced in four of 210 separate infusions. Data are presented to support the use of IVIG in the management of childhood autoimmune disorders.
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PMID:Efficacy of intravenous gamma globulin in autoimmune-mediated pediatric blood dyscrasias. 311 7


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