Gene/Protein Disease Symptom Drug Enzyme Compound
Pivot Concepts: Gene/Protein Disease Symptom Drug Enzyme Compound   Target Concepts: Gene/Protein Disease Symptom Drug Enzyme Compound

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Query: UMLS:C0021051 (immunodeficiency)
71,517 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Some cases of idiopathic thrombocytopenic purpura (ITP) may be related to the prodromal phase of the acquired immunodeficiency syndrome (AIDS). The morphologic features of six spleens removed in this context were studied. In the white pulp, lymphoid follicular hyperplasia was constant but hardly distinguishable from that of control ITP on routine light microscopic grounds. Moreover, its bland appearance contrasted with the major histopathologic changes usually described in lymph node biopsy specimens from patients with AIDS-related complex. Immunohistochemistry and electron microscopy demonstrated, respectively, an excess of cytotoxic-suppressor lymphocytes with a decreased CD4/CD8 ratio in the germinal centers and viral particles of the human immunodeficiency virus. The immunohistochemical and ultrastructural studies were more helpful than light microscopy in presenting histopathologic arguments in favor of the human immunodeficiency virus infection.
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PMID:Idiopathic thrombocytopenic purpura in patients at risk for acquired immunodeficiency syndrome. Histopathologic study, immunohistochemistry, and ultrastructural study on six spleens. 326 46

Intravenous immune globulin (IV-IgG) is approved for use in substitution therapy of immunodeficiency syndromes and treatment of idiopathic thrombocytopenic purpura (ITP). The initial dose for substitution therapy is 0.2 g/kg body weight repeated monthly. If necessary, the dose may be increased to 0.3 g/kg and the frequency to every 2-3 weeks. The approved dosage for ITP is 0.4 g/kg daily for 2 to 5 consecutive days. Repeat doses of 0.4 g/kg have been used as maintenance therapy. The initial infusions of IgG to immunodeficient patients who have not been treated during the previous 8 weeks or never treated at all should proceed with caution. These patients are at risk of potentially serious complement-mediated adverse reactions. Adverse reactions due to IgG administration are less frequent and less severe for patients with ITP. Currently available preparations are contraindicated in patients with a selective IgA deficiency and detectable IgA antibodies.
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PMID:Intravenous immune globulin: dosage and administration. 329 97

Intravenous immunoglobulins are stable monomeric pooled human IgG preparations for therapeutic use. Three intravenous immunoglobulins licensed in the United States are generally therapeutically equivalent. Intravenous immunoglobulin is the preferred agent for replacement therapy for most patients with primary or secondary antibody immunodeficiency because of the rapidity and ease of giving large quantities of IgG, even by self-administration. Disadvantages of intravenous immunoglobulins include frequent (approximately 10%) but usually not serious side effects, the need for venous access (often difficult in infants and young children), and high cost. Intravenous immunoglobulins are also beneficial in the prevention of certain viral infections, such as cytomegalovirus pneumonia and varicella; they may also have a synergistic effect with antibiotics in certain bacterial diseases. Intravenous immunoglobulin has also been used successfully in the management of idiopathic thrombocytopenia purpura, Kawasaki disease, and certain autoimmune diseases. Intravenous immunoglobulin may also be of use in certain high-risk and premature newborns.
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PMID:Intravenous immunoglobulins as therapeutic agents. 330 51

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

This study included 36 children with IgA-deficiency, increased susceptibility to infections and/or other disorders. Recurrent, usually bacterial infections were noticed in 23 out of 26 patients (88%) with complete and in 7 out of 10 patients (70%) with partial IgA-deficiency. All patients with severe infections had complete IgA-deficiency. Complete IgA-deficiency was also present in the six children who had autoimmune disorders associated with recurrent infections. In 22 out of the 36 patients studied the serum could be analysed for concomitant IgG subclass deficiencies: one patient had marked decrease of IgG2. In a second patient IgG4 was not detectable. Two patients had combined IgG2-IgG4-deficiency. In a girl with severe acute and chronic infections and relapsing idiopathic thrombocytopenic purpura, IgA-IgG2-IgG4-deficiency was found to be the prodromal stage of common variable immunodeficiency with panhypogammaglobulinaemia.
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PMID:Susceptibility to infections in children with selective IgA- and IgA-IgG subclass deficiency. 376 75

To our knowledge, only six families with familial idiopathic thrombocytopenic purpura (ITP) have been described in the literature. We encountered two elderly siblings with ITP, which normally occurs only in younger persons. It has been clearly demonstrated that ITP is an autoimmune disorder, and there is some evidence that it reflects an inherited immunodeficiency.
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PMID:Idiopathic thrombocytopenic purpura in two elderly siblings. 407 42

Human immunodeficiency virus 1-related idiopathic thrombocytopenic purpura (HIV-1-ITP) patients have a 4-fold increased percentage of CD5+ B cells and a 4.8-fold increased percentage of serum immune complexes precipitated by polyethylene glycol (PEG-ICs) compared to control subjects, as reported previously. Since CD5+ B cells produce predominantly IgM rheumatoid factor (RF) vs. Fc of IgG and PEG-ICs contain high levels of IgM, we looked for the presence of RF in the immune complexes of HIV-1-ITP patients. PEG-ICs were adsorbed to protein A and dissociated with acid, and IgM and IgG were purified by gel filtration and affinity chromatography. Solid-phase ELISA was used to measure antibody specificity vs. platelets, Fc, and HIV-1 gp120, p24, and CD4. Dissociated IgG antibody reacted with platelets, HIV-1 gp120, p24, and CD4, but not with Fc. Serum IgG did not react with platelets or Fc but did react with HIV-1 gp120, p24, and CD4. Both PEG-IC IgM and serum IgM reacted with Fc as well as the other four antigens. Control IgM and IgG were unreactive. Isolated IgM from PEG-ICs relocated approximately 50% of the IgG preincubated with IgM to the Vo region of a G200 gel-filtration column. Anti-platelet IgG but not IgM could be affinity-purified from fixed platelets. Both F(ab')2 fragments of anti-platelet IgG and the total PEG-IC bound to platelets in a saturation-dependent manner. F(ab')2 of anti-platelet IgG inhibited 50% binding of PEG-IC to platelets at an F(ab')2/complex ratio of 3:1 (wt/wt). Scatchard analysis revealed two classes of binding sites: high-affinity Kd values of 0.8-1.8 nM and lower-affinity Kd values of 6.6-12.3 nM with respective numbers of binding sites of 44,000-57,000 and 122,000-256,000 (n = 4). Anti-platelet IgG of 6/6 patients precipitated GPIIIa from platelet lysates of surface 125I-labeled platelets. Platelet count correlated inversely with anti-platelet IgG (r = -0.73; P < 0.01; n = 27). Thus, PEG-ICs of HIV-1-ITP patients contain IgM RF, which sequesters serum anti-platelet IgG containing anti-GPIIIa. Anti-platelet IgG contributes to binding of immune complexes to platelets and correlates with thrombocytopenia.
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PMID:Sequestration of anti-platelet GPIIIa antibody in rheumatoid factor immune complexes of human immunodeficiency virus 1 thrombocytopenic patients. 789 59

Selective tropism of human immunodeficiency virus (HIV) for cells with CD4 receptors and especially for TH lymphocytes--key cells in hematopoiesis--has from the clinico-biologic point of view a great many hematologic manifestations of which knowledge is essential for a good diagnosis and treatment as well as for a judicious estimation of prognosis. Thus the study presents the hematologic entities specifically associated with HIV infection (such as ITP, NHML) as well as the hematologic entities associated with HIV infection without presenting a causal relationship with the latter. The study also discusses the quantitative and morphologic changes of peripheral blood and of bone marrow often enlightening for the disease and its complications. An important chapter in the study is devoted to the hematologic changes induced by the HIV infection therapy as well as by the manners of therapeutic approach to the complex hematologic problems raised by the disease.
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PMID:Hematologic manifestations of infection with the human immunodeficiency virus. 792 Mar 33

The main immunological abnormality in human immunodeficiency virus (HIV)-infected patients, and particularly those with the acquired immune deficiency syndrome (AIDS), is a deficiency in cellular immunity. However, symptomatic HIV-infected children also have evidence of deficiency of specific antibody synthesis, and intravenous immune globulin (IVIG) preparations in doses of 0.2-0.4 g/kg every 2-4 weeks have been shown to reduce the incidence of respiratory infections. IVIG therapy may also reduce the mortality and incidence of bacterial infections in adults but further studies are required. In addition, high-dose IVIG therapy (1-2 g/kg over 2-5 days) produces increased platelet counts in patients with idiopathic thrombocytopenic purpura (ITP) associated with HIV infection. Finally, IVIG therapy may have a role in HIV-infected patients suffering from severe parvovirus B19 or measles infection, or in patients suffering from autoimmune disorders where high-dose IVIG therapy has been shown to be efficacious.
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PMID:Does intravenous immune globulin have a role in HIV-infected patients? 803 37

A morphometric and immunohistochemical study was performed to assess the spleen's alterations in patients with autoimmune thrombocytopenia and in drug abusers with thrombocytopenia (DAT) related to human immunodeficiency virus (HIV) infection. A total of 34 patients were included in the study: 20 DAT patients and 14 with idiopathic thrombocytopenic purpura (ITP). Twenty HIV-negative splenectomy patients without thrombocytopenia were included as controls. Spleen weight in DAT patients (323.25 +/- 149.96 g, mean + standard deviation) was significantly increased compared with the ITP (164.28 +/- 29.79 g, P < 0.0001) and control (175.50 +/- 49.14 g, P < 0.0001) groups. The mean diameter of lymphoid follicles in the spleens of DAT patients (446.83 +/- 99.16 microns, was significantly higher than in those of the control patients (370.87 +/- 55.30 microns, P = 0.019). In control patients' spleens, the number of platelets in Billroth's cords was significantly higher (59.54 +/- 32.72/10(4) microns 2) than in those of the DAT (2.13 +/- 1.42/10(4) microns 2, P < 0.0001) and ITP (P < 0.0001) patients. The number of macrophages and ceroid histiocytes per 10(4) microns 2 of red pulp was significantly increased in both DAT (5.14 +/- 1.90) and ITP (7.48 +/- 4.38) patients compared with the control patients (3.66 +/- 1.10, P < 0.0001) and P = 0.06, respectively), and in ITP patients compared with DAT patients (P = 0.0136). The number of granulopoietic precursors per 10(4) microns 2 of red pulp was higher in the spleens of DAT (1.41 +2- 1.46, P < 0.0001) and ITP (0.92 +/- 0.75, P < 0.0001) patients compared with those of the control group. Transmission electron microscopy studies demonstrated platelet phagocytosis by macrophages of Billroth's cords and presence of myeloid metaplasia in spleens of DAT and ITP patients. Immunohistochemical studies showed a depletion of CD4+ lymphocytes in the T zone of splenic white pulp and an increased number of CD8+ lymphocytes in red pulp of DAT patients' spleens compared with those of ITP and control patients. There were no significant alterations in dendritic reticular cell network in the DAT group compared with the ITP and control groups.
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PMID:Comparative study of spleen pathology in drug abusers with thrombocytopenia related to human immunodeficiency virus infection and in patients with idiopathic thrombocytopenic purpura. A morphometric, immunohistochemical, and ultrastructural study. 824 10


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