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Query: UMLS:C0002874 (aplastic anemia)
 5,905 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Environmental control in managing patients with immunodeficiency ranges from the exceedingly complex to the relatively simple. At one end of the spectrum is the total isolation technology applied to David, the "Bubble Boy" who lived his entire life behind sterile plastic barriers. At the other end of the spectrum is the simpler technology applied to patients receiving bone marrow transplants who are maintained in ordinary private hospital rooms and attended by personnel who merely observe handwashing precautions. Most properly performed and controlled studies of the use of special isolation procedures to reduce infections derive from patients receiving bone marrow transplants for conditions of aplastic anemia and leukemia or patients receiving chemotherapy for malignancy. The design of isolation procedures for immunodeficient patients borrows from these studies because of the relatively small number of immunodeficient patients. These studies have shown that laminar airflow rooms produce a significantly lower incidence of infections but may not change the mortality of all patients. Also, protective isolation has clearly reduced the incidence and severity of graft-versus-host disease in transplanted patients with aplastic anemia. Recently there has been a trend away from strict isolation procedures because careful studies have indicated that host rather than acquired pathogens are responsible for at least 85% of infections in these special patients. Also, the human stress of prolonged isolation is becoming increasingly recognized. The complex and expensive isolation techniques that were used in David's case are no longer being utilized in immunodeficient subjects, partly because new transplantation technology has made it possible to cross histocompatibility barriers, obviating the need for permanent isolation.
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PMID:Environmental control in management of immunodeficient patients: experience with "David". 352 65

To estimate the cellular immune response of workers highly exposed to mixtures of organic solvents, subpopulations of peripheral blood lymphocytes (PBLs) were analyzed. For this, the PBLs of nine floorers (aged 25-58 years, exposure time 8-35 years) were subsequently labelled with monoclonal antibodies OKT4, OKT8, OKT11, anti-Leu 7 and anti-Leu 12. Analysis was made by a FACS IV cell sorter (Becton-Dickinson, USA). The control group consisted of matched pairs of healthy donors. In the exposed group we found a decrease in the OKT11 (all) T cell fraction, a decrease in the OKT4 helper cells, an increase in the anti-Leu 7 positive cells, mostly natural killer cells, an important increase in anti-Leu 12 labelled T cells, i.e., human B-lymphocytes, and no differences in the OKT8 suppressor cells. Total fluorescence intensity profiles between the exposed and the unexposed group did not differ, i.e., the marker density on the cell surfaces remained unchanged. Similar changes in lymphocyte subpopulations are found in states of immunodeficiency and immunogenetic forms of aplastic anemia, a disease whose etiological relationship may be due to long-term exposure to organic solvents.
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PMID:Lymphocyte subpopulations in solvent-exposed workers. 394 94

Analyses of 100 subjects with the X-linked lymphoproliferative syndrome (XLP) in 25 kindreds revealed four major interrelated phenotypes: infectious mononucleosis, malignant B-cell lymphoma, aplastic anemia, and hypogammaglobulinemia. Eighty-one of the patients died. Two male subjects were asymptomatic but showed immunodeficiency to Epstein-Barr virus (EBV). Seventy-five subjects had the infectious mononucleosis phenotype and concurrently, 17 subjects of this group had aplastic anemia. All subjects with aplastic anemia died within a week. Aplastic anemia did not accompany hypogammaglobulinemia or malignant lymphoma phenotypes. Hypogammaglobulinemia had been detected before infectious mononucleosis in three subjects, after infectious mononucleosis in five subjects, and was not associated with infectious mononucleosis in 11 boys with hypogammaglobulinemia. In nine subjects infectious mononucleosis appeared to have evolved into malignant lymphoma; however, the majority of patients with malignant lymphoma showed no obvious antecedent infectious mononucleosis. One subject had infectious mononucleosis following recurrent malignant lymphoma. Twenty-six of 35 lymphomas were in the terminal ileum. Results of immunologic and virologic studies of 15 survivors revealed combined variable immunodeficiency and deficient antibody responses to EBV-specific antigens. Mothers of boys with XLP exhibited abnormally elevated titers of antibodies of EBV. Subjects of both sexes with phenotypes of XLP should be investigated for immunodeficiency to EBV. Persons with inherited or acquired immunodeficiency may be vulnerable to life-threatening EBV-induced diseases.
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PMID:Epstein-Barr virus-induced diseases in boys with the X-linked lymphoproliferative syndrome (XLP): update on studies of the registry. 628 85

The human immune system has evolved multiple cellular and humoral defense mechanisms against the lymphotropic virus, EBV. NK cells, suppressor T-cells, cytotoxic K-cells, memory T-cells, and humoral immune responses usually subdue the virus into latency. Individuals with immune deficiency are at great risk of developing immunoregulatory disturbances and lymphoproliferative diseases when confronted by EBV. The infection of B-cells by EBV provokes a marked activation of immunoregulatory T-cells and requires restoration of immune homeostasis during convalescence. This is accomplished with difficulty in an individual with significant immune defects. The X-linked lymphoproliferative syndrome is an exemplary model for studying EBV in immune deficient individuals. Boys with XLP can develop acquired agammaglobulinemia, aplastic anemia, chronic or fatal IM, and a variety of B-cell malignant lymphomas following infection by the virus. We have identified multiple immune defects in the patients and progressive immunoregulatory disturbances following infection by the virus. Other patients with immune deficiency syndromes, i.e., ataxia telangiectasia or the renal transplant recipient, are also at increased risk for developing EBV-induced lymphoproliferative diseases. Moreover, certain families are at increased risk for EBV-associated malignancies, especially those with a triad of manifestations (i.e., autoimmunity, immunodeficiency, and lymphoma). Chromosomal breakage as seen in patients with ataxia telangiectasia may predispose to leukemogenesis. Immunoregulatory defects are also probably predisposing factors to lymphomagenesis. Both inherited and acquired defects can render persons vulnerable to leukemia and lymphoma.
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PMID:Immunodeficiency as a factor in lymphomagenesis. 633 Jun 65

Marrow grafting, once undertaken only after failure of all other forms of therapy, is now the preferred therapy for some malignant diseases. Chemoradiotherapy and marrow grafting for patients with acute leukemia who have failed chemotherapy results in cure rates of 10%-30%. For patients under the age of 50 with acute nonlymphoblastic leukemia transplanted in first remission, the cure rate is approximately 50% with better results in younger patients. Marrow grafting is now being explored in a variety of types of malignant diseases having in common a steep dose-response curve to therapy, therapy limited by marrow toxicity, and the availability of a suitable marrow donor. Current research in the field of marrow transplantation is reviewed and provides a basis for a reasonable expectation that results of marrow transplantation will continue to improve. The use of partially matched family members or phenotypically histocompatibility leukocyte antigen-identical unrelated donors will make marrow grafting available to a larger fraction of patients. Marrow grafting, developed for the treatment of malignant disease, has found an important application to nonmalignant diseases, including immunodeficiency syndromes, aplastic anemia, and thalassemia and other genetic disorders of hematopoiesis.
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PMID:Karnofsky Memorial Lecture. Marrow transplantation for malignant diseases. 636 43

We assessed the number of Langerhans cells (LC) before and after bone marrow transplantation (BMT) in 27 patients in order to study the fate and behavior of these dendritic antigen-presenting cells following allogeneic BMT. LC were identified using monoclonal antibody OKT6 on skin biopsies performed on days - 10, 0, 11, 25, 39, 120, and 365. In a control group composed of 15 healthy adults aged 20-37 yr, the mean number of LC (+/- SEM) was 25.6 +/- 1.17/0.1 sq mm of epidermal surface. Our study shows that pretransplant, the number of LC in patients with aplastic anemia or leukemia was lower than that of controls. The finding of low numbers of LC in patients with untreated aplastic anemia is suggestive of a medullary origin of LC in man. Moreover, during the early posttransplant period, nearly all patients present a severe deficit in LC. This deficit may delay the maturation of their immune system. The number of LC reaches nearly normal levels 4-12 mo after BMT. Finally, we have noted a significant impairment of LC reconstitution in patients with acute graft-versus-host disease (GVHD), providing evidence that this defect may be an important mechanism involved in acute GVHD-related immunodeficiency.
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PMID:Study of Langerhans cells after allogeneic bone marrow transplantation. 636 51

Extensive immunologic studies were done in 97 patients with severe aplastic anemia between 1973 and 1979. Sixteen young male patients with hepatitis-associated aplastic anemia appeared to constitute a unique subset. Compared with most patients with aplastic anemia from other causes, these 16 patients had significant reductions in the mean values of circulating T lymphocytes, serum IgG and IgM, mitogen reactivity, and decreased cutaneous hypersensitivity. The ratio of peripheral blood helper to suppressor T lymphocytes identified by monoclonal antibodies was within normal limits in 3 patients studied with hepatitis-associated aplastic anemia. Interestingly, the ratio was low (less than 1) in 3 of 7 patients studied with aplastic anemia from other causes, although the mean for these 7 patients was normal. These data suggest that patients in this subset with hepatitis-associated severe aplastic anemia have a severe immunodeficiency. Whether this immunodeficiency is the cause or result of the hepatitis or aplastic anemia, or both, is unknown.
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PMID:Immunologic defects in young male patients with hepatitis-associated aplastic anemia. 660 93

The X-linked lymphoproliferative syndrome (XLP) is characterized by a combined variable immunodeficiency with vulnerability to Epstein-Barr virus (EBV)-induced fatal or chronic infectious mononucleosis, acquired agammaglobulinemia, aplastic anemia, or malignant B cell lymphomas. Diagnosis of XLP requires documentation of two or more maternally related males with these phenotypes. Epstein-Barr virus must be demonstrated in circulating blood, lymphoid tissues, or saliva of infected males. Characteristically, the patients have low-titer antibodies to EBV and often lack anti-EB nuclear-associated antibody due to T cell defects. Thymus gland is often depleted and epithelium may be destroyed. Thymic-dependent regions in lymph nodes and spleen are depleted and immunoblastic transformation with plasma cell differentiation is seen. The carrier females exhibit partial immune deficiency and have paradoxically elevated antibodies to EBV. Our registry of XLP provides consultation and comprehensive study of persons and families with the syndrome.
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PMID:X-linked lymphoproliferative syndrome. An immunodeficiency disorder with acquired agammaglobulinemia, fatal infectious mononucleosis, or malignant lymphoma. 689 75

Patient survival after BMT is directly correlated with the HLA-type of the donor. The survival rate after BMT from an HLA-genotypically identical sibling is 56% in acute leukemia, 55% in combined severe immunodeficiency disease (SCID) and 67/83% in severe aplastic anemia patients. The usage of only HLA-D identical related or unrelated donors in SCID revealed a 37% survival, compared to 18% survival in acute leukemia and 11% in severe aplastic anemia using HLA-phenotypical identical or HLA-D identical related donors. BMT from HLA-phenotypical and MLC identical unrelated donors resulted in death of the grafted patients. Non of the patients grafted with HLA-different marrow survived BMT. Survival of BMT patients depended beside the histocompatibility matching on the clinical treatment and the clinical constellation of the patient: The survival rate decreased in aplastic anemia patients due to sensibilisation caused by pre-BMT blood transfusion and was significantly higher in leukemia when BMT was performed in remission.
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PMID:[HLA and bone marrow transplantation (BMT) (author's transl)]. 702 87

Cellular immune functions were evaluated longitudinally in seven children with severe aplastic anemia, who were successfully transplanted with bone marrow cells from an HLA-identical, mixed lymphocyte culture (MLC)-negative sibling. Several parameters were followed: the number of lymphocytes and E rosette-forming cells in the peripheral blood and the lymphocyte reactivity toward various mitogens, antigens, and allogeneic lymphocytes. Some patients already displayed decreased in vitro lymphocyte reactivity before transplantation, especially with regard to the response to pokeweed mitogen (PWM). After transplantation, a severe cellular immunodeficiency developed in all patients, with low numbers of T cells and markedly impaired responsiveness to mitogens, antigens, and allogeneic lymphocytes. Variations between patients were substantial, both with regard to the severity and duration of the immunodeficiency and to the pattern of the recovery of lymphocyte responses to mitogens and antigens. This variability might be attributable to an imbalanced proliferation of different lymphocyte subsets and/or the sequence of appearance of receptors for mitogens on the cell surface.
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PMID:Bone marrow transplantation in children with severe aplastic anemia: reconstitution of cellular immunity. 703 58


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