Gene/Protein Disease Symptom Drug Enzyme Compound
Pivot Concepts:   Target Concepts:
Query: UMLS:C0018133 (graft-versus-host disease)
 18,032 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

A three-month-old girl with congenital immune deficiency developed graft-versus-host disease following engraftment of maternal immunocompetent cells. T and B lymphocyte numbers increased and lymphocyte responsiveness to phytohemagglutinin normalized during the patient's hospitalization. However, these cells failed to respond to pokeweed mitogen and several specific antigens, suggesting that the expanding clone of alloreactive cells had limited immunologic potential. Serum IgE concentration rose from an undetectable level to 2,600 u/ml, indicating an immunoregulatory imbalance. HLA typing revealed that the patient's parents shared HLA antigen specificities. Finally, experimental administration of antithymocyte globulin had no beneficial effect upon the patient's clinical course or laboratory findings.
 ...
PMID:Partial immunologic reconstitution and hyperimmunoglobulinemia-E in neonatally-acquired graft-versus-host disease. 696 44

Three recipients of HLA-identical bone marrow transplants developed chronic graft-versus-host disease (cGVHD) and hypergammaglobulinemia. All three had evidence of abnormal B-lymphocyte function, including a polyclonal increase in immunoglobulins (Ig), antinuclear antibodies, rheumatoid factor, lymphocytotoxins, and increased immune complexes. T-lymphocyte function was also abnormal, including decreased mitogen reactivity and delayed cutaneous hypersensitivity. The cellular basis of these immune abnormalities was studied in an in vitro system in which we analyzed spontaneous pokeweed mitogen (PWM) driven Ig synthesis. Multiple defects in both T- and B-lymphocyte function were detected. In contrast to normal B cells, circulating B cells from all three patients with cGVHD spontaneously synthesized in vitro greater than 200 ng of IgG and in two of the three greater than 175 ng of IgM. This increase in spontaneous Ig synthesis was not due to a deficiency of regulatory cells, since T cells from the three patients suppressed spontaneous Ig synthesis in a normal fashion. In contrast to this increased spontaneous Ig synthesis, the response of the patients' B cells to PWM-driven Ig synthesis was normal. Using the PWM system we demonstrated several defects in these patients' T cells, including increased suppressor activity and decreased helper cell activity. These data indicate that some patients with cGVHD have multiple defects in both T- and B-cell function that may contribute to their profound immune deficiency.
 ...
PMID:Lymphocyte dysfunction in chronic graft-versus-host disease. 697 20

Possible explanations for the immune deficiency usually associated with graft-versus-host disease include defective lymphocyte differentiation and active suppressor cell mechanisms. The present observations in a patient with chronic graft-versus-host disease delineate yet another possible mechanism: a failure of successful interaction between competent patient lymphocytes. Thus, helper and suppressor T cells as well as precursor B cells for the generation of specific in vitro plaque-forming cell (PFC) responses were present among patient lymphocytes and could interact normally with cells from four unrelated normal donors and produce PFC responses. In contrast, patient cells failed to interact successfully with each other and generate PFCs. This suggests a highly (self-) specific block in cell interaction that could explain the inability of the patient to mount a PFC response in vitro as well as a number of antibody responses in vivo during this phase of his disease.
 ...
PMID:Failure of T and B cell cooperation during graft-versus-host disease. 701

Injection of parental lymphocytes into an unirradiated adult F1 host results in an acute GVH reaction characterized by immune deficiency, attack on host lymphohematopoietic tissues, and repopulation with donor-derived cells. All of these events result from the initial activation of donor lymphocytes by host alloantigens. Interaction of pairs of host and donor costimulatory molecules, in particular CD28/CTLA4 and B7-1/B7-2, play a crucial role in this initial activation of donor T cells. We demonstrate here that in vivo treatment of the host with high doses of CTLA4-Ig solely during the initial period of donor alloactivation can completely abort the subsequent development of GVH reaction. Although donor T cells are retained, CTLA4-Ig treatment reduces the initial endogenous cytokine production and arrests the subsequent expansion of donor T cells, the differentiation of anti-host effectors, and the development of severe immune deficiency. This result is consistent with the establishment of host-specific tolerance in the donor population, while maintaining host immune competence.
 ...
PMID:Acute graft-versus-host reaction can be aborted by blockade of costimulatory molecules. 763 32

We have reviewed the causes and risk factors for early death in a group of 295 children who underwent any form of first bone marrow transplantation (BMT) between 1978 and 1992. The commonest indications for transplantation were acute lymphoblastic leukaemia 80 (27.1%), neuroblastoma 69 (23.3%), immune deficiency 57 (19.3%) and myeloid leukaemias/myelodysplasia 50 (16.9%). There were 120 (40.6%) allogeneic BMTs, 118 (40%) autologous BMTs, while 51 (17.2%) children usually with severe combine immune deficiency received BMT from a non-HLA-identical parent, sibling or other relative (FBMT). Two were from identical twins and four from matched unrelated donors (MUD). Thirty-three children (11.2%) died in the first 100 days; the main causes of death being infection (n = 5), relapse (n = 7), graft failure (n = 4), GVHD (n = 7) and organ failure with or without infection (n = 6). There was no significant change in the incidence of early deaths in the three successive 5 year periods (1978-82, 1983-87, 1988-92) although there was some shift in the causes. Infections were the commonest cause during the first 5 year period, relapses followed by GVHD in the second period and single organ failure followed by GVHD and infections in the third period. The main causes of early death were relapse after high-dose chemo/radiotherapy and autologous BMT (7 of 9 deaths) and GVHD and infection after allogeneic BMT (9 of 13 deaths). In the group of 51 children undergoing FBMT there were five deaths from infection, three from graft failure, one from organ failure and one from GVHD.(ABSTRACT TRUNCATED AT 250 WORDS)
 ...
PMID:Early deaths in children undergoing marrow ablative therapy and bone marrow transplantation. 771 76

Transfusion-associated graft-versus-host disease (TA-GVHD) is a rare but lethal disorder caused when viable donor lymphocytes engraft and proliferate in a susceptible transfusion recipient. Patients with immune deficiency disorders, hematologic malignancies and bone marrow transplants are at risk to TA-GVHD, as are premature newborns and transfusion recipients who are HLA heterozygous for an HLA-haplotype that is shared with an HLA homozygous donor. Irradiation of blood components with 2500 cGy will inactivate donor lymphocytes and prevent TA-GVHD. Platelets and granulocytes are not functionally impaired by this radiation dose, but red cells sustain detectable damage. Red cell units irradiated and stored for 42 days have significantly higher supernatant recovery of chromium-51 labeled cells is sub-optimal. Based on these data, the maximum permissible storage time for irradiated red cells has been reduced to 28 days.
 ...
PMID:Transfusion-associated graft-versus-host disease and the irradiation of blood components. 771 2

Graft versus host disease is frequently encountered in patients with an allogenic bone marrow transplantation. The disease apparently results from the activity of the donor T lymphocytes which react against the recipient's cells. There are two categories of graft versus host disease: the acute form which occurs within 3 months of the graft and the chronic form which occurs thereafter. Skin is the predominant site of manifestations, although the liver, the gut and the eye may be involved. In acute graft versus host disease, there is a characteristic maculo-papular rash raising a difficult differential diagnosis which pathology examination of biopsy cannot always resolve. The chronic disease is easier to recognize on the basis of local or generalized lichenoid or sclerodermal skin lesions. Several risk factors have been identified including the degree of donor-recipient HLA mismatch, recipient age and the number of T cells grafted. Graft versus host disease leads to immune deficiency with lymphoid depletion and increased risk of infection. Treatment depends on the site of organ involvement and extension. Corticosteroids and immunosuppressors are used.
 ...
PMID:[Cutaneous graft versus host disease]. 777 Apr 16

A patient without evident immune deficiency who received a transfusion of blood from a second-degree family member developed fatal transfusion-associated graft-versus-host disease (TA-GVHD). The donor was homozygous for an HLA haplotype for which the recipient was heterozygous (one-way HLA match). All 39 reported cases of TA-GVHD in immunocompetent patients were reviewed to ascertain the predisposing factors and to define the indications for irradiating blood for this population. HLA typing was described in 15 cases; in 13, including seven related and six unrelated donors, a one-way HLA match was present. Thirty-one (79%) of the 39 cases were reported from Japan (and 196 other cases are cited in the Japanese literature), but a one-way HLA match among unrelated donors at HLA-A, -B, -DR loci is only approximately two to four times more likely in Japanese persons than in whites. Fresh blood (< 96 hours old) was used in 29 (94%) of the 31 cases reported from Japan and in 33 (87%) of 38 cases overall (in one case, the age of the blood used was not reported). Thus, factors that appear to predispose to TA-GVHD in immunocompetent patients are a one-way HLA match, fresh blood, and, possibly, Japanese ancestry. Irradiating cellular blood components from all blood relatives of transfusion recipients will not completely eliminate the risk of TA-GVHD.
 ...
PMID:Transfusion-associated graft-versus-host disease in immunocompetent patients: report of a fatal case associated with transfusion of blood from a second-degree relative, and a survey of predisposing factors. 821 11

Allogeneic bone marrow transplantation is concerned by immunology by at least two aspects: the first one is the acceptance of the graft by the host and reciprocally and the second one is that it constitutes an unique human model of immune reconstitution. In this review of the immunological aspects, we deal with the selection of the bone marrow donor (related or not) especially on the base of HLA compatibility and the graft-versus-host disease (GVH) with the clinical manifestations, the usual treatments, the supposed cellular mechanisms and the risk factors of developing such complications. The graft versus leukemia effect (GVL) which may be linked to the GVH disease and the mechanisms of rejection and take of the graft are also reviewed as well as the immune reconstitution following the immune deficiency due to the conditioning treatment and the occurrence of a GVH disease.
 ...
PMID:[Immunologic aspects of bone marrow transplantation]. 835 46

With a prevalence of approximatively 50%, diarrhoea is a frequent event in immune deficiency of any cause. Because this condition is permanent in AIDS, the main characteristic of diarrhoea is chronicity. Non infectious causes are more common in conditions other than AIDS with, for exemple, intestinal injuries related to graft versus host disease or to chemotherapy toxicity. Among infectious causes, enteric parasitic diseases such as cryptosporidiosis or microsporidiosis are more commonly observed in the immunodeficiency related to HIV while viral infections due to cytomegalovirus or adenovirus seem possible in any case of severe troubles of the immunity. Gram-negative infections and Clostridium difficile colitis have to be diagnosed because efficient treatment is available. In these patients usually in bad general condition, because diarrhoea is often of unknown aetiology or non curable, the diagnostic and therapeutic strategy has to be pragmatic in order to control the symptoms without an excess of invasive procedures.
 ...
PMID:[Diarrhea in immune deficiency status]. 874 35


<< Previous 1 2 3 4 5 6 7 8 9 10 Next >>