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)

A prospective study of IgG and IgM isotypes of anticardiolipin antibodies (aCL) was performed in a series of 167 patients with various autoimmune diseases, including rheumatic and nonrheumatic disorders, and in a group of 100 healthy blood donors. The IgG aCL serum was regarded as positive if a binding index (BI) greater than 2.85 (3.77 SD) was detected and a BI greater than 4.07 (3.90 SD) was defined as positive for IgM aCL. Forty patients (24%) were found to be positive for IgG and/or IgM aCL. IgG aCL were detected in 23% of patients with systemic lupus erythematosus (SLE), in 9% with idiopathic thrombocytopenic purpura, in 7% with progressive systemic sclerosis, and in 6% with dermatomyositis-polymyositis. IgM aCL were present in 43% patients with primary biliary cirrhosis, in 33% with rheumatoid arthritis, in 22% with SLE, and in 8% with giant-cell arteritis. IgG aCL were found to have a significant association with thrombosis and thrombocytopenia, and IgM and aCL with haemolytic anaemia and neutropenia, in SLE but not in the other autoimmune diseases. The identification of these differences in the aCL isotype associations, depending on the autoimmune disorder, may improve the clinical usefulness of these tests.
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PMID:Anticardiolipin antibodies in patients with autoimmune diseases: isotype distribution and clinical associations. 261 16

Morphologically distinct lymphoid cells with homogeneous, condensed chromatin and scant cytoplasm can be observed in large numbers in the bone marrow of children with a variety of hematologic and nonhematologic disorders. In some patients, these cells may account for greater than 50% of the bone marrow cells, creating a picture that can be confused with acute lymphoblastic leukemia (ALL) or metastatic tumor. Although originally called hematogones (HGs), a variety of other names have been proposed for these unique cells. The clinical significance of expanded HGs has not been resolved, and the biologic features of these cells are incompletely described. In this study, we correlate the clinical, morphologic, cytochemical, flow cytometric, molecular, and cytogenetic properties of bone marrow samples from 12 children with substantial numbers of HGs (range 8% to 55% of bone marrow cells). Diagnoses in these patients included anemia, four; neutropenia, one; anemia and neutropenia, one; idiopathic thrombocytopenic purpura, two; retinoblastoma, two; Ewing's sarcoma, one; and germ cell tumor, one. Flow cytometric analyses of bone marrow cells demonstrated a spectrum extending from early B-cell precursors (CD10+, CD19+, TdT+, HLA-Dr+) to mature surface immunoglobulin-bearing B cells in these patients, corroborating our morphologic impression of HGs, intermediate forms, and mature lymphocytes. DNA content was normal, and no clonal abnormality was identified by either cytogenetic or immunoglobulin and T-cell receptor (TCR) gene rearrangement studies. Follow-up ranged from 3 months to 3 years. None of the patients has developed acute leukemia or bone marrow involvement by solid tumor. The possible role of HGs in immune recovery and hematopoiesis is presented.
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PMID:Hematogones: a multiparameter analysis of bone marrow precursor cells. 291 89

The number of reports about successful therapeutical application of 7S-immunoglobulins in various auto-immune diseases such as ITP or autoimmune neutropenia or in myasthenia gravis has increased in recent years. Multiple Sclerosis (MS) is an inflammatory demyelinating disease, in which antibodies directed against various components of the myelin sheath play an important pathogenetic part. Increased antibody dependent cytotoxicity has been reported in MS, and myelinotoxic antibodies and a myelination inhibiting factor have been found in acute cases. Lately several authors have tested 7S-immunoglobulins in the treatment of MS. It is still too early for final judgement of the therapeutical efficacy, however, patients suffering from the progressive form of MS with exacerbations showed rather fast improvement of their latest neurological deficits, when treated shortly after a fresh bout. Mechanisms of action which should be taken into consideration include reduced antibody dependent cytotoxicity by surface receptor blocking reduction of the number of circulating myelinotoxic antibodies caused by a negative feed back mechanism, and removal of a neuroelectric inhibiting factor caused by antibodies.
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PMID:[Gamma globulin therapy in multiple sclerosis. Theoretical considerations and initial clinical experiences with 7S immunoglobulins in MS therapy]. 294 24

Natural killer cell (NK) activity was assessed in patients before and after treatment with intravenously administered immune globulin (IVIG). In eight patients with hypogammaglobulinemia or agammaglobulinemia receiving 300 mg/kg/dose IVIG every 4 weeks, NK activity was significantly lower after therapy than before. In two patients, one with idiopathic thrombocytopenic purpura and one with autoimmune neutropenia, receiving high doses (2 gm/kg) of IVIG, NK activity was unusually high before therapy. After treatment, NK activity decreased in correlation with the clinical response and elevation of peripheral cell counts. These data show that IVIG diminishes NK activity in vivo and that reduction of NK activity may be associated with clinical improvement in idiopathic thrombocytopenic purpura and autoimmune neutropenia. NK activity of lymphocytes obtained from healthy volunteers was reduced by the same concentrations of maltose or sucrose present in Gamimune or Sandoglobulin, respectively; IVIG preparations, however, were more inhibitory. The diminution of NK activity therefore may be related to two components of IVIG preparations, monomeric IgG and maltose or sucrose.
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PMID:Effect of intravenous immune globulin on natural killer cell activity: possible association with autoimmune neutropenia and idiopathic thrombocytopenia. 308 May 73

Six patients had serious complications as consequences of treatment of idiopathic thrombocytopenic purpura. Five had splenectomy-related complications, one of them developed fatal intra-abdominal bleeding. Three patients acquired operation-related serious infection, two of them died. Serious neutropenia after vinblastine-loaded platelets occurred in one patient leading to pseudomonas septicaemia and panophthalmitis with permanent vision loss of left eye. Recurrence thrombocytopenia occurred in every case during serious complications. Early detection by awareness of the possibility of serious complications can reduce morbidity and mortality occurring after therapy of idiopathic thrombocytopenic purpura.
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PMID:Serious complications following treatment of chronic idiopathic thrombocytopenic purpura. 321 19

Idiopathic thrombocytopenic purpura (ITP) is a disorder characterized by a low platelet count and purpura. Identification of an antiplatelet antibody suggests that this is an autoimmune disease. Corticosteroids and splenectomy have been the major therapies for many years. High-dose intravenous immune globulin (IV-IgG) has been very successful in the management of ITP and has recently received FDA approval for this condition. It was also successful when administered to a few patients with autoimmune neutropenia. The exact mechanism of action of IV-IgG in autoimmune disorders is poorly understood. Currently, high-dose IV-IgG has a definite place in the management of ITP. Its role in therapy includes emergency treatment, preoperative preparation, the postponement of splenectomy in young children, and treatment of ITP during pregnancy.
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PMID:Immunotherapy of idiopathic thrombocytopenic purpura and autoimmune neutropenia. 329 1

Between February 1983 and April 1986 we studied peripheral blood and bone marrow samples from 20 patients with human immunodeficiency virus (HIV) related disease. 14 patients had AIDS, three had ARC, two had PGL and one had ITP as a sole manifestation of HIV related disease. Peripheral blood abnormalities included marked anisocytosis and poikilocytosis, rouleaux formation, neutropenia, lymphopenia, monocytopenia, a left shift in the granulocyte series and, in the patients with AIDS, vacuolated monocytes. The most frequent bone marrow abnormalities were reticuloendothelial iron block, dyserythropoiesis, megaloblastic change and erythroid hypoplasia. Excess histiocytes were noted in four marrows, one exhibiting haemophagocytosis. None of the bone marrows showed lymphopenia. Eight of the 20 marrows were difficult or impossible to aspirate. None of the trephine biopsies showed increased reticulin. The causes of these abnormalities are probably multiple and include opportunistic infections, drug therapy, immune mechanisms and possibly direct insult by the HIV virus.
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PMID:Peripheral blood and bone marrow abnormalities in patients with HIV related disease. 356 82

Intravenous immunoglobulin (IVIG) may be considered first-line maintenance therapy for idiopathic thrombocytopenic purpura (ITP) because it has been proven to be the least toxic. In a study of 25 children with acute ITP, treatment with IVIG maintained platelet counts above 40,000/mm3 in all of the children. After 1 year, none of these patients required further therapy. In another study group of 25 pediatric patients with chronic ITP, treatment with IVIG circumvented splenectomy in 60% of the cases. The therapeutic regimens for adults and children are described, as is a strategy to overcome IVIG resistance. Experience with IVIG in hemolytic anemia and neutropenia are discussed. The mechanism of action is explored in some detail, specifically as it relates to reticuloendothelial system (RES) Fc receptor blockade and suppression of antiplatelet antibody synthesis.
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PMID:Therapy in cytopenia. 376 52

High-dose intravenous immunoglobulin is being used increasingly as a new therapeutic approach towards various autoimmune diseases, yielding encouraging results predominantly in idiopathic thrombocytopenic purpura (ITP). A marked initial increase of the platelet count has been observed in the majority of patients with acute and chronic ITP alike after high-dose immunoglobulin. In contrast to acute ITP, where full or partial remissions have been achieved in nearly all cases, long-term results reached in chronic ITP, where lasting increases of platelet counts have been observed predominantly in splenectomized patients, have proved to be less favourable. Correspondingly, several cases of other forms of autoimmune thrombocytopenia and neutropenia successfully treated by high-dose immunoglobulin have been reported. We contribute our own experience in 4 patients with myasthenia gravis, where administration of 7S-immunoglobulin but not of 5S-immunoglobulin was followed by both a clinical remission as well as a decrease of specific autoantibody concentration. While several mechanisms of action of high-dose immunoglobulin are discussed, there is evidence for an immunosuppressive effect and for the Fc-fragment dependency of the therapeutic efficacy. Although high-dose immunoglobulin has proved to be a promising therapeutic option especially in ITP, indications will have to be defined yet by further investigations and may be restricted to emergency and refractory cases.
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PMID:[High-dose immunoglobulin therapy in autoimmune diseases]. 608 2

The rapid rise in platelet count after immunoglobulin treatment in acute and chronic forms of idiopathic thrombocytopenic purpura (ITP), autoimmune neutropenia, and post-transfusion purpura is well documented. It is suggested that the rise in platelet count is due to competitive inhibition of the macrophage binding of platelets by preferential sequestration of immunoglobulin-coated red blood cells. Measurement of haptoglobin levels, a sensitive indicator of haemolysis, suggests that clinically inapparent haemolysis occurs during immunoglobulin therapy of ITP patients. In-vitro experiments confirm that there is immunoglobulin coating of red blood cells. The hypothesis is further supported by the findings that immunoglobulin treatment in autoimmune haemolytic anaemia is ineffective, and that platelet counts rise in some ITP patients after induction of a mild haemolytic syndrome by injection of anti-Rho (D).
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PMID:Effect of intravenous immunoglobulin in immune thrombocytopenia. 613 31


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