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)

In summary, intravenous gammaglobulin appears to be a very useful treatment of immune haematologic disease. Almost all patients with ITP treated with IVIg will have an increase in their platelet counts lasting at least 2 weeks. Some of these patients will derive long-term benefit from the IVIg and many can maintain their platelet counts by periodic single outpatient infusions. Patients with immune neutropenia are also likely to benefit by IVIg treatment which may be of particular benefit due to its not interfering with phagocyte function. RES blockade is a well-documented mechanism of action immediately after IVIg infusion and platelet antibody synthesis may decrease in some patients; IVIg does not appear to protect platelets from platelet antibodies.
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PMID:The use and mechanism of action of intravenous immunoglobulin in the treatment of immune haematologic disease. 636 4

Between 1956 and 1981, 306 splenectomies for hematologic diseases were performed at the UCLA Medical Center. Of these operations, more than 75% were performed for therapeutic reasons to control anemia, thrombocytopenia, neutropenia, or painful symptoms of splenomegaly. Of the 65 patients who had idiopathic thrombocytopenic purpura, 77% showed an excellent response, and of the 39 patients who had hereditary spherocytosis, 90% responded. Other diseases with predictably good response rates were autoimmune hemolytic anemias, Felty's syndrome, and hairy cell leukemia. Forty patients with Hodgkin's disease had splenectomies for diagnostic purposes the last 10 years. The overall morbidity and mortality were 24% and 6%, respectively, the most common complications being pneumonia, wound infections, and local postoperative bleeding, and the most common cause of death being sepsis. The review supports the thesis that in carefully selected patients, therapeutic splenectomy can have desirable palliative effects and that diagnostic splenectomy has a sufficiently low risk to warrant its consideration in patients with Hodgkin's disease.
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PMID:Splenectomy for hematologic disease. The UCLA experience with 306 patients. 673 25

A relation between lymphoma and autoimmune neutropenia, unlike autoimmune hemolytic anemia and idiopathic thrombocytopenic purpura, has not previously been well documented. We report a patient with a disorder presenting as autoimmune agranulocytosis, splenomegaly, and infiltration of the hepatic sinusoids by lymphocytes. Antineutrophil antibodies were present. Over a 2 1/2-year period, the illness progressed to an aggressive, poorly differentiated lymphocytic lymphoma with terminal liver failure and fibrosis. Peripheral blood lymphocyte markers identified the tumor as a proliferation of T-cells of the helper class. A review of previous literature disclosed other reports of similar patients who had neutropenia, a lymphoproliferative illness, and hepatic disease. Our case is representative of a previously unrecognized syndrome characterized by autoimmune neutropenia in the setting of a lymphoproliferative disorder of T cells, with a predilection for liver involvement.
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PMID:Lymphoma with autoimmune neutropenia and hepatic sinusoidal infiltration: a syndrome. 698 Jun 14

The combination of idiopathic thrombocytopenic purpura and autoimmune hemolytic anemia is rare in childhood. Among 164 instances of ITP and 15 instances of AHA, 11 patients were found to have this combination. Three were found to have systemic lupus erythematosus, one had aplastic anemia, and seven had Evans syndrome. Neutropenia, at times associated with bacterial infections, occurred in four of the latter patients. Unlike most cases of ITP or AHA in childhood, the clinical course of Evans syndrome is usually chronic and relapsing. Treatment including corticosteroids, splenectomy, and immunosuppressive agents has been generally unsatisfactory. In view of the frequent presence of antibodies directed at red blood cells, platelets, neutrophils, and lymphocytes, immunopancytopenia may be a better term for this condition.
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PMID:Evans syndrome in childhood. 719 90

High-dose intravenous gammaglobulin has been successfully used in several autoimmune diseases such as idiopathic thrombocytopenic purpura, autoimmune neutropenia; more recently this treatment has been experimented in other autoimmune conditions with conflicting results. After a review of recent literature, the article considers results obtained by high-dose intravenous gammaglobulin therapy in some conditions such as connective tissue diseases and vasculitis. A review of different mechanisms of action hypothesized in the different disorders is reported. In fact, it is quite clear today that this treatment has not only clinical effects but achieves also measurable immunological and biological results so that we can consider high-dose gammaglobulin as a immunomodulating treatment.
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PMID:[Experimental treatment with high-dose gamma globulins in autoimmune diseases]. 750 33

Autoimmune thrombocytopenic purpura (AITP) is generally a chronic disorder in affected adults. Twenty-five percent of these patients will become refractory to routine therapy (corticosteroids and splenectomy), as well as most other available agents. Intravenous pulse cyclophosphamide therapy was used to treat 20 patients with severe refractory AITP who had previously failed to achieve a sustained remission with a mean of 4.8 agents (range 2 to 8). Patients received 1 to 4 doses (mean 2.0) of 1.0 to 1.5 g/m2 intravenous cyclophosphamide per course. Of the 20 patients treated with pulse cyclophosphamide therapy, 13 patients (65%) achieved a complete response (CR), four (20%) a partial response (PR), and three patients (15%) failed to respond. Of the 13 complete responders, eight have remained in remission with stable platelet counts during followup intervals of 7 months to 7 years (median 2.5 years). Five patients developed recurrent AITP 4 months to 3 years following a CR. Of these, two patients responded to subsequent courses of pulse cyclophosphamide therapy with current remissions of 1 and 4 years. Of the four patients who obtained a PR, two remain in partial remission after 10 months and 4 years; one relapsed after 18 months and, after retreatment, is still in remission at 6 months. Of the patient characteristics examined, duration of disease was most strongly associated with response to pulse cyclophosphamide. Side-effects of treatment included neutropenia (three patients, one of whom developed staphylococcal sepsis), acute deep venous thrombosis (two patients), and psoas abscess (one patient). Intravenous pulse cyclophosphamide should be strongly considered in the treatment of patients with refractory AITP. There is a relatively low incidence of side-effects, and it can be administered easily on an out-patient basis.
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PMID:Pulse cyclophosphamide therapy for refractory autoimmune thrombocytopenic purpura. 779 54

A series of 213 neutropenic patients were tested for the presence of granulocyte antibodies using the granulocyte chemiluminescence test (GCLT) and the granulocyte immunofluorescence test (GIFT). Sera containing lymphocyte (HLA) antibodies were excluded from the study. A direct GIFT was performed on granulocytes from 56 patients. Samples were obtained from patients with a range of clinical conditions including primary adult autoimmune neutropenia, autoimmune neutropenia of infancy, autoimmune neutropenia secondary to Felty's syndrome, rheumatoid arthritis, idiopathic thrombocytopenic purpura, systemic lupus erythematosus, proliferative disorders, bone marrow transplantation and patients with documented febrile or pulmonary transfusion reactions. Overall, granulocyte antibodies were detected in 52.1% of patient sera. Results for the GCLT and GIFT (IgG) were strongly correlated (p < 0.001) for both primary and secondary immune neutropenias. The results confirm the applicability of the GCLT in the granulocyte serology laboratory.
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PMID:Prospective evaluation of the chemiluminescence test for the detection of granulocyte antibodies: comparison with the granulocyte immunofluorescence test. 818 97

In the last years high dose intravenous immunoglobulin (i.v.IG) has clearly modified the therapeutic approach toward autoimmune hemocytopenia. Their introduction has promoted the studies on the i.v.IG mechanisms of action: the hypothesis most widely accepted is the blockade of Fc-receptors in the reticuloendothelial system. It was hypothesized that low levels of anti-red blood cell antibodies contained in the i.v.IG preparations might be responsible for the Fc-receptor blockade. On this basis, anti-Rh(D) immunoglobulin has been successfully tested in Rh-positive patients with idiopathic thrombocytopenic purpura (ITP). The present paper describes the presumed mechanisms of action, clinical indications, side effects and cost of the anti-Rh(D) immunoglobulin. Until now, anti-Rh(D) has been chiefly employed in patients with ITP and human immunodeficiency virus-related ITP. However, at present, new clinical indications are emerging from studies in patients with autoimmune neutropenia, both using the intravenous and the intramuscular route. Anti-Rh(D) immunoglobulin has proved, in our and in other Authors' experience, a safe and easy to be administered treatment, at low cost and slightly lower in efficacy compared with i.v.IG.
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PMID:[Anti-Rh(D): an efficacious therapeutic alternative in autoimmune hemocytopenias]. 826 53

Besides cytopenia related to treatment, several hematological disorders such as anemia, abnormal platelet activity, thrombosis, presence of anticardiolipin or anti-neutrophil antibodies, cyclic neutropenia, and myelodysplasia, have been reported in patients with Crohn's disease (CD). The case we report here is the first one documenting the association of idiopathic thrombocytopenic purpura (ITP) with CD.
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PMID:Idiopathic thrombocytopenic purpura associated with Crohn's disease. 954 29

Neutropenia is frequently observed in a variety of autoimmune disorders. As the mechanism of neutropenia in these disorders, the destruction of neutrophils by anti-neutrophil autoantibodies has been believed since elevated levels of neutrophil-associated IgG (NAIgG) have been described. However, no data exists to characterize the nature of NAIgG and show NAIgG is an anti-neutrophil autoantibodies. We investigated whether the elevated NAIgG in these patients consists of anti-neutrophil autoantibodies. The NAIgGs of 91 patients with autoimmune disorders including 50 patients with idiopathic thrombocytopenic purpura, 13 patients with systemic lupus erythematosus, 11 patients with Hashimoto's thyroiditis and 10 patients with Graves' disease were analyzed. The level of NAIgG was high in 36 of 91 patients. Elution studies were performed to determine whether NAIgG has a nature of autoantibodies. In model experiments, the ether eluate from neutrophils sensitized with neutrophil-specific alloantibody (anti-NA2) reacted with donor neutrophils, whereas the eluates from those with model immune complexes (ICs) failed. These data indicated that the ether elution technique is useful to determine whether NAIgG consists of anti-neutrophil autoantibodies. The NAIgG on patient's neutrophils was eluted with ether and the reactivity of the eluate with normal neutrophils was investigated. The eluates from 34 of 36 patients with various autoimmune disorders with elevated NAIgG level failed to react with donor neutrophils. These data indicated that the elevated NAIgG in the majority of these patients did not consist of anti-neutrophil autoantibodies, but possibly of ICs.
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PMID:Characterization of elevated neutrophil-associated IgG in various autoimmune disorders: not anti-neutrophil autoantibodies, but possibly immune complexes, bind to neutrophils. 955 Feb 87


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