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Query: UMLS:C0018133 (graft-versus-host disease)
 18,032 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

A 50-year-old severely immunodeficient woman with malignant non-Hodgkin lymphoma died from graft-versus-host disease due to transfusion of a single unit of packed red cells. Three days after this transfusion a maculo-papular rash appeared, followed by generalized erythroderma refractory to therapy and eventually progressing into generalized ulcero-squamous dermatitis. This case, and a review of other similar cases published elsewhere, prompt the authors to recommend prophylactic irradiation of blood products prior to their administration to patients with cellular immunodeficiency, particularly in cases of acute leukaemia or malignant lymphoma where patients receive intensive radio- and/or chemotherapy regimens. To appreciate the degree of cellular immunodeficiency in such risk patients, simple criteria should be developed to assess the efficiency of the cellular immune system.
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PMID:[Acute graft-versus-host disease following a single transfusion of erythrocytes]. 396 40

Two patients with acute leukemia were treated with chemoradiotherapy and allogeneic bone marrow transplantation. Despite the prophylactic use of methotrexate after grafting, both patients developed severe graft-versus-host disease that was refractory to treatment with methylprednisolone. The graft-versus-host disease was then treated with a monoclonal antibody, 64.1, that reacts with a p19 antigen on human T cells. The disease responded dramatically to this treatment, but both patients subsequently developed a fatal polyclonal lymphoproliferative disorder arising in donor-derived B cells. Hybridization studies showed Epstein-Barr virus in both tumors. The combined effect of severe end-stage graft-versus-host disease and potent immunosuppressive therapy probably resulted in a progressive immunodeficiency syndrome that abrogated the T-cell-mediated surveillance mechanism that normally modulates the proliferation of Epstein-Barr-virus-infected B lymphocytes.
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PMID:Fatal Epstein-Barr-virus-associated proliferation of donor B cells after treatment of acute graft-versus-host disease with a murine anti-T-cell antibody. 608 3

In one and one-half decades, marrow transplantation has become a life-saving procedure for an ever-increasing number of patients. With continued observation, however, it became apparent that marrow transplantation as used presently is associated with significant acute, delayed, and chronic toxicity. These complications may be related to chemo- or radiotherapy used in conditioning for transplantation, to immunosuppressive treatment given after transplantation and to the immunodeficiency associated with transplantation and the development of GVHD. Attempts need to be made to develop less toxic conditioning regimens. Most importantly, however, we need to learn more about mechanisms of graft-host interaction in order to eliminate the problem of GVHD and to accelerate the recovery of immunocompetence in allogeneic chimaeras.
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PMID:Bone marrow transplantation: a review of delayed complications. 632 56

Recipients of allogeneic bone marrow transplants are characterized by an immunodeficiency of varying intensity and duration. We have previously demonstrated the presence of in vivo activated suppressor T lymphocytes in immunodeficient patients with chronic graft-versus-host disease. To determine the basis of the immunodeficiency of transplant recipients early after transplantation, the lymphocytes of transplant recipients were analyzed phenotypically by E-rosette formation and staining with monoclonal antibodies (OKT-3, -6, and -8) and functionally by their blastogenic response to mitogens. Only 15% of transplant recipients' assays 0-3 months and 16% of assays 3-12 months following transplant were in the normal range. Transplant recipients during the first year after transplantation were characterized by an increased percentage (57%) of patients with a normal percentage of E-rosette-forming cells but reduced PHA responsiveness. In vitro coculture experiments demonstrated that their lack of PHA responsiveness was not due to the presence of in vivo activated suppressor cells or a decrease in mitogen-presenting cells. Staining with monoclonal antibodies revealed that the T lymphocytes from the majority of recipients at 0-3 months following transplantation contained a percentage of OKT8-positive cells greater than or equal to the percentage of OKT3-positive cells. This pattern (OKT8 greater than or equal to OKT3) was not found in the peripheral blood T lymphocytes of normal people but was found in 13 of 15 thymuses. Monoclonal staining with OKT6, a thymocyte-specific antibody, revealed positive staining of more than 10% of the peripheral blood leukocytes in the majority of recipients 0-3 months following transplantation, compared with only a few normals. We concluded that the circulating T lymphocytes of transplant recipients are phenotypically and functionally immature, and that their relative immaturity contributes to the transplant recipients' immunodeficiency.
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PMID:Immature T lymphocytes in the peripheral blood of bone marrow transplant recipients. 636 45

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

Two infants with immunodeficiency with predominant T-cell defects received transplants of HLA-identical bone marrow cells along with thymopoietin pentapeptide (TP-5) treatment and no prior immunosuppressive therapy. Both patients achieved durable engraftment with early reconstitution of cell-mediated immunity. The study of cell surface antigens with monoclonal antibodies (MoAb) revealed that the early appearance of T-cell subsets defined by OKT4 and OKT8 MoAb occurred. Neither of the patients showed any signs or symptoms of graft versus host disease over a 1-year period. This experience suggests that patients with T-cell deficiency who do not benefit from thymic hormones alone can be successfully treated by bone marrow transplantation. The association of TP-5 with bone marrow transplantation seems to induce an early and stable reconstitution and to protect against fatal post-transplant infection.
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PMID:Bone marrow transplantation and thymopoietin pentapeptide treatment in two infants with immunodeficiency with predominant T-cell defects. 638 71

The ability to define subpopulations of immunologically competent lymphocytes has permitted an enhanced understanding of the interaction between functionally distinct components of the immune system. T cells can provide help in antibody formation or they may suppress antibody production. Abnormal immunoregulatory mechanisms have been demonstrated in the hyperimmunoglobulin E-recurrent infection syndrome. This disorder is associated with a marked elevation of IgE and specific elevations of IgE antibodies directed toward staphylococcal antigens. Abnormal T cell regulation of immune responses has been demonstrated. Graft-versus-host disease (GVHD) occurs in an immunodeficient patient who has received an infusion of immunocompetent cells. The diagnosis of graft-versus-host (GVH) reaction may be complicated by the protean manifestations of the disorder. The acute form, consisting of a maculopapular rash, fever, and diarrhea, may be confused with acute infection or drug reaction. Chronic GVHD has been incorrectly diagnosed as histiocytosis X, acrodermatitis enteropathica, or scleroderma. Utilizing chromosome markers and/or identification of histocompatibility antigens, the presence of circulating lymphocytes from donor immunocompetent cells (blood transfusion, maternal source) can be documented. The development of sensitive technics for identifying cells can establish a precise diagnosis. Certain immunodeficiency disorders can be identified by biochemical means. Biotin-dependent multiple carboxylase enzyme deficiency is associated with a chronic dermatitis, alopecia, ataxia, and secondary infection of the skin with Candida. The disorder responds promptly to the administration of biotin with correction of dermatologic, neurologic, and immunologic abnormalities.
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PMID:New insight into the causes of immunodeficiency disorders. 638 1

Impairment in T cell proliferation in response to E. coli and in CML to unrelated alloantigens was usually observed in patients early after marrow grafting and persisted in long-term patients with chronic GVHD but not in those without chronic GVHD. We analyzed various cellular functions in the pathway of T cell activation and found that in patients with immunodeficiency, (1) their M phi usually could process and present antigens to normal T cells, (2) their T cells did not proliferate even in the presence of normal antigen-pulsed M phi, (3) IL-2 production by T cells was deficient, and (4) exogenous IL-2 restored CML activity in cells of most patients early after grafting but not in cells of most patients with chronic GVHD. Therefore, failure to induce proliferation and cytotoxicity in T cells of marrow recipients is not likely due to M phi defects but because of ineffective communication among T cell subsets, probably related to impaired IL-2 production and/or unresponsiveness to IL-2.
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PMID:Ineffective cellular interaction and interleukin 2 deficiency causing T cell defects in human allogeneic marrow recipients early after grafting and in those with chronic graft-versus-host disease. 639 Aug 48

Graft versus host disease (GVHD) is a well recognized entity following bone marrow transplantation. Similar syndromes have been described after blood product transfusions, notably in patients with primary immunodeficiency syndromes and in patients with malignancies associated with immune deficiency or under immunosuppressive treatment. Review of the literature shows that posttransfusion GVHD is characterized by maculopapular skin rash, gastro-intestinal symptoms, liver disease, severe pancytopenia and, in some cases, hepatosplenomegaly and lymphadenopathy. The time to onset and the duration of the disease are short (10 days) and the mortality approaches 90%. The clinical features of this rare disorder are presented in the hope that, with increased awareness of this complication, clinicians will take preventive measures in patients at risk because no satisfactory therapy yet exists.
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PMID:[Clinical characteristics and evaluation of risk in the graft versus host reaction following transfusion]. 663 42

The case of a 20-year-old man with lymphoblastic lymphoma in leukemic phase succumbing to acute graft-versus-host disease (GVHD) following intensive chemotherapy and blood transfusions is described. Such a documented association has rarely been reported in the literature. The issue of irradiating blood components prior to transfusion in patients with suspected cell-mediated immunodeficiency receiving intensive chemotherapy is raised and discussed.
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PMID:Graft-versus-host disease in lymphoblastic lymphoma following blood transfusions. 668 10


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