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)

Cyclophosphamide (Cy), an alkylating agent widely used in chemotherapy of leukemia and cancer, causes a well-documented toxicity on hematopoietic and lymphoid cells. Neutropenia is thought to be the main factor involved in infectious complications following antimitotic chemotherapy. Little is known on the effects of these therapies on the mucosal associated lymphoid system which is one of the main barriers against environmental pathogenic agents. The present study examined the effects of a single administration of Cy (200 mg/kg) on murine T and B cell populations of Peyer's patches (PPs), IgA secretion in the proximal part of the small intestine, and plasma cells of the lamina propria. Cy induced in mice a transient decrease in the T and B cell populations of the PPs with a drastic fall of B cell counts and a profound decrease of intestinal IgA secretion due to a reduction of lamina propria plasma cells. This transient secretory IgA deficiency may contribute to the infectious complications following antimitotic chemotherapy.
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PMID:Transient secretory IgA deficiency in mice after cyclophosphamide treatment. 195 41

Immune defense mechanisms play an essential protective role against infections caused by a wide array of pathogenic microorganisms. Although the growing number of the available antimicrobial agents has certainly improved the overall clinical outcome of such infections, antimicrobial therapy not rarely fails whenever the host's immune function is depressed. On the other hand, recently introduced therapeutic and diagnostic procedures (antineoplastic chemotherapy causing severe neutropenia and mucositis; organ transplantation requiring conditioning regimens; the widespread use of intravascular catheters and prosthetic devices; administration of adrenal corticosteroids and/or other immunosuppressive agents) have resulted in an unprecedented number of immunocompromised hosts. In addition, a variety of antibiotics have been found to display adverse effects on specific and non-specific immune functions, thus further impairing the already depressed immune system of the host. Antibiotic-mediated immunomodulation hence is explored with the introduction of a third-generation cephalosporin, namely cefodizime (CDZ), which has been shown to possess immunostimulating properties in preliminary in vitro and ex vivo studies as well as in a few experimental animal models. A chronoimmunopharmacological approach to CDZ-induced immunomodulation has been started by ourselves. The study, which is still in progress, includes patients with multiple myeloma (MM), selective IgA deficiency and chronic uremia, and matched healthy subjects. A number of immunological parameters are being assessed on blood samples drawn every 6h in the 24-h span prior to CDZ administration (a single 2 g daily dose i.v. for 6 days to the patients and for 4 days to healthy subjects), and in the 24h following the last CDZ injection. Healthy subjects and patients are randomly assigned to two groups, depending on whether they are given the antibiotic at 0800 or at 1800. Although a full evaluation of the results will be reported elsewhere, the group of MM now includes 24 patients. A circadian stage-dependent chronoimmunomodulating effect has been unequivocally shown for the monocytic chemotactic responsiveness to CDZ in MM. Immunostimulating 'side-effects' suggest that CDZ should possibly be regarded as a prototype antimicrobial agent for patients with impaired immune functions. Conceivably, a better knowledge of such properties will help synthesize new antibiotics with specific immunomodulating effects.
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PMID:Antimicrobial agents as biological response modifiers (BRM) and chrono-immunomodulation: an emerging relationship. 304 51

Ninety-one congenitally immunodeficient patients treated from 1972 to 1981 were reviewed to assess the incidence and nature of gastrointestinal complications. Thirty-three of these patients (36%) developed 59 complications. Patients with immunodeficiencies characterized by neutrophil dysfunction--chronic granulomatous disease (20 patients) and cyclic neutropenia (eight patients)--developed 22 surgical infections, 22 of which required operation. In patients with neutrophil defects, postoperative morbidity was frequent and severe. Gastrointestinal symptoms were common in patients with isolated defects of B or T lymphocytes. Ten of forty-one patients with congenital hypogammaglobulinemia developed gastrointestinal complications, as did one of four patients with DiGeorge Syndrome, and the single patient with secretory IgA deficiency. However, operation was not required for these patients with isolated disorders of lymphocyte function. Patients with combined B and T cell disorders developed gastrointestinal disease, requiring operative therapy at intermediate rates. Gastrointestinal symptoms developed in four of nine patients with severe combined immunodeficiency and three of eight with Wiskott-Aldrich syndrome. Operative therapy was required in two of these seven symptomatic patients.
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PMID:Gastrointestinal complications of congenital immunodeficiency states. The surgeon's role. 660 28

We report an infant with autoimmune neutropenia (AIN), idiopathic thrombocytopenia (ITP), and IgG2/IgA deficiency. The patient was referred to our hospital at 5 months of age because of epistaxis and generalized petechiae. Physical examination revealed moderate hepatosplenomegaly. A complete blood count revealed a platelet count of 2.0 x 10(3) cells/microliters, and a white cell count of 3,600 cells/microliters, with severe neutropenia (less than 1% bands and segmented cells). Neutrophils and platelets adhering to megakaryocytes were decreased in the bone marrow. Tests for serum neutrophil-binding IgG (NB-IgG) and platelet-associated IgG (PA-IgG) were positive. A diagnosis of both AIN and ITP was made and therapy with intact-type gamma-globulin and prednisolone was initiated. Improvement occurred, but was temporary. A lack of serum IgA and IgG2 was noted during the clinical course. The patient has not been susceptible to bacterial infections but has had a severe clinical course with rubella and chickenpox.
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PMID:An infant with both autoimmune neutropenia and idiopathic thrombocytopenia with IgG2/IgA deficiency. 847 60

Common variable immunodeficiency (CVI) is a primary immunodeficiency characterized by deficient antibody production. The cause of this immunodeficiency is unknown; several in vitro studies have revealed a significant number of alterations that could explain the hypogammaglobulinemia present in this syndrome. Among those described are primary B cell alterations, numerical and functional T cell abnormalities, and defects in the interaction between accessory cells. The alteration typical of CVI is the failure of B lymphocytes to differentiate from antibody-producing cells, resulting in deficient immunoglobulin secretion. Among the T cell abnormalities described are a diminished proliferative response to mitogens and antigens, alterations in the level of production of several cytokines, especially reduction in the production of IL-2, diminished antigen-specific T cells and increase basal apoptosis after stimulation. Antigen presenting cells, monocytes and dendritic cells can also present alterations and contribute to deficient antigen response. The clinical manifestations of these patients is variable; most present recurrent bacterial infections due to encapsulated bacteria, especially sinusitis, otitis, bronchitis, and pneumonias. A few patients can present mycobacterial or fungal infection and occasionally Pneumocystis carinii. Viral infection is uncommon in these patients although some suffer recurrent herpes zoster infection. Clinical features of septicemia and central nervous system infections are less frequent. The incidence of digestive tract infections in these patients is high. The most common cause of diarrhea is Giardia lamblia; Salmonella, Shigella and Campylobacter are also common pathogens. Autoimmune disease is also more prevalent in these patients than in the general population. The most frequently associated diseases are hemolytic anemia, idiopathic thrombocytopenic purpura and autoimmune neutropenia. Cancer is also frequently associated with CVI, the most common forms being lymphoproliferative syndromes, especially non-Hodgkin's lymphoma. Granulomas are a unusual manifestation in some patients with CVI; their localization varies but the most commonly affected organs are the spleen and lungs. Some authors have compared these granulomas with those characterizing sarcoidosis, especially when appearing in the lung. Diagnosis of CVI is usually by exclusion of other diseases, such as cystic fibrosis, immotile cilia syndrome or allergic processes. CVI should be suspected in all patients with recurrent bacterial infections especially those localized in the respiratory tract. Other primary immunodeficiencies which present clinical findings similar to CVI and which should be ruled out are selective IgG subclass deficiency, IgA deficiency and selective deficiency in the response to polysaccharide antigens with normal immunoglobulin levels. The serum hypogammaglobulinemia present in all patients with CVI provides the diagnostic key. The age at which clinical manifestations appear, the absence of familial antecedents and the presence of circulating B lymphocytes form the basis of the differential diagnosis between X-linked agammaglobulinemia and autosomal recessive forms. The treatment of choice of patients with CVI is treatment with human gamma-globulin. Currently, the most common route of administration is intravenous; these molecules have a half-life of approximately 21 days and a high degree of safety concerning the possible transmission of viral infections. Adverse reactions are generally few and clinically unimportant. The most frequently used doses oscillate between 200 and 400 mg/kg body weight every 2-4 weeks. Both the dose and its frequency should be personalized for each patient. Early diagnosis of patients with CVI, application of treatment with appropriate antibiotics for infections and treatment with gamma-globulins prevent long-term complications of this disease and dramatically improve the quality of life and life expectancy of these patients.
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PMID:[Common variable immunodeficiency. Review]. 1143 84

The primary immunodeficiency diseases are a heterogeneous group of more than 75 disorders characterized by intrinsic defects in the functions of the immune system. Many are associated with abnormalities of hematopoiesis as well. This article will review those primary immunodeficiency syndromes in which neutropenia is a prominent finding, including X-linked agammaglobulinemia (XLA), hyper IgM syndrome, common variable immunodeficiency (CVID), IgA deficiency, cartilage-hair hypoplasia (CHH), and reticular dysgenesis, with regards to pathophysiologic findings and treatment.
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PMID:Neutropenia associated with primary immunodeficiency syndromes. 1195 93

IgA deficiency is a relatively common congenital immunodeficiency in children. It can either be asymptomatic or lead to frequent infections, most often of the sinuses and lungs. Intensive chemotherapy for acute leukemia is also profoundly immunosuppressive and can be complicated with life-threatening infections, usually associated with neutropenia and prolonged lymphopenia in the post-bone marrow transplant setting. Isolated, acquired immunoglobulin deficiency that occurs during treatment has been described but is usually transient. In this report, the authors describe a patient with infant acute myelogenous leukemia with acquired, persistent IgA deficiency.
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PMID:Isolated IgA deficiency after chemotherapy for acute myelogenous leukemia in an infant. 1463 25

Recurrent respiratory infections (RRIs) are a common and benign condition affecting about 6% of schoolchildren. Only mild, likely postinfective, modifications of the immune system have been proven, and parents should be reassured that the condition is self-limited. Nevertheless, if not correctly diagnosed, children may undergo several unnecessary investigations and multiple antibiotic courses. On the other hand, in some cases, efforts should be made to identify promptly possible underlying disease, including congenital or acquired immunodeficiency, vascular or airways malformation, tuberculosis, cystic fibrosis, or immotile-cilia syndrome. Careful medical history and clinical examination are usually sufficient to distinguish RRIs and no further research is generally needed. In uncertain cases a complete blood count with differential and the evaluation of total immunoglobulin serum levels are sufficient to exclude neutropenia, T- or B-lymphocyte defects, and selective IgA deficiency. It is essential to observe environmental risk factors: reducing environmental tobacco smoke at home is a fundamental goal and the postponed enrolment of children at day-care centres reduces the risk of RRIs. Antibiotic treatment are not justified since they do not shorten the course of the condition or prevent complications. Use of anti-cough syrups should be avoided. Nasal lavage with saline serum and the blowing are the only justified interventions. Adenoidectomy and tonsillectomy should be planned only in conditions included in validated guidelines.
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PMID:Recurrent respiratory infections: why not talking about it any more? 1897 2

Patients with congenital immunodeficiency (CID) syndromes are susceptible to various microorganisms. However, relatively few CID disorders develop mycobacterial disease. We describe clinical features, laboratory findings and therapeutic outcome of children with CID who had tuberculosis disease. Medical reports of 10 patients were reviewed. Three patients had chronic granulomatous disease, two had common variable immuno deficiency, the others had cyclic neutropenia, combined immunodeficiency, hyperimmunoglobulin E syndrome, selective IgA deficiency and X-linked agammaglobulinemia. Eight patients presented with pulmonary tuberculosis, one had tuberculosis arthritis, one had tuberculosis osteomyelitis. There was acid fast bacilli in sputum of two, bone marrow aspiration in one and postmortem lung biopsy specimen in one patient. Mycobacterium tuberculosis grew in sputum of one and articular fluid aspirate of one patient. One patient was diagnosed with bone biopsy specimens characteristic for tuberculosis. The remaining three patients were diagnosed to have tuberculosis disease as they had positive tuberculin skin test and clinical and radiologic findings unresponsive to non-specific treatment. All patients were treated with antituberculous drugs. Mycobacterium species may be important pathogens in children with CID, especially in endemic regions.
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PMID:Tuberculosis in children with congenital immunodeficiency syndromes. 2051 30