Gene/Protein Disease Symptom Drug Enzyme Compound
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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)

We report a rare disease of skin and oropharyngeal mucosa in a 28-year-old patient occurring 2 years after an allogeneic bone marrow transplantation. The dermatologic diagnosis was unambiguously epidermolysis bullosa acquisita according to the immunofluorescence and clinical presentation. Treatment with cyclosporin A and prednisone resulted in resolution. This autoimmune skin disease may be a manifestation of graft-versus-host disease, but the relationship must remain speculative.
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PMID:Epidermolysis bullosa acquisita, a rare late complication of allogeneic bone marrow transplantation? 157 12

Lichenoid dermatosis is a pattern description of a variety of cutaneous lesions which primarily affect the dermoepidermal junction. Involvement of skin appendages has been restricted to hair follicles in lichen planopilaris and discoid lupus erythematosus. Sweat gland involvement has not been described in the four common members of this group, namely, lichen planus, discoid lupus erythematosus, fixed drug eruptions and erythema multiforme, although structural abnormalities have been reported in graft-versus-host disease. In a detailed morphological study of 59 cases, including lichen planus (12), discoid lupus erythematosus (18), fixed drug eruption (14) and erythema multiforme (15), 78% (47/59) showed sweat, gland abnormalities. The abnormalities included vacuolation of cell cytoplasm, with and without lymphocytic infiltration, apoptosis of basal cells and basal cell hyperplasia of the excretory ducts which predominantly affected the portion of the duct adjoining the acrosyringium. The portion of the duct close to the secretory gland was only involved in continuity and the secretory glands were unaffected. These abnormalities of the sweat gland mostly constitute primary involvement by the disease process in contrast to structural abnormalities secondary to fibrosis.
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PMID:Sweat gland abnormalities in lichenoid dermatosis. 183 38

We have reviewed results of secondary therapy in 427 patients with acute graft-versus-host disease (GVHD) who did not have a durable satisfactory response after primary treatment. At the beginning of secondary treatment, 320 patients (75%) had rash, 252 (59%) had liver dysfunction, and 228 (53%) had gut dysfunction. Secondary treatment was with glucocorticoids (n = 249), cyclosporine (n = 80), antithymocyte globulin (n = 114), or monoclonal antibody (n = 19) either singly (n = 390) or in combination (n = 37). Parameters of GVHD severity were recorded weekly, and responses were determined according to values at the initiation of tertiary treatment or, for patients without such treatment, using values on day 29 of secondary treatment or the last recorded values before death, whichever occurred first. Minimal criteria for improvement or deterioration were defined for each organ, but no attempt was made to define liver or gut outcome if another complication such as venocclusive disease or infectious enteritis was present. Improvement or resolution of GVHD in the respective organ was seen in 45% of patients with skin disease, 25% of patients with evaluable liver disease, and in 35% of patients with evaluable gut disease. Overall complete or partial responses were seen in 40% of patients. The highest complete response rate with secondary therapy (23%) was seen when GVHD recurred during the taper phase of primary glucocorticoid treatment and was managed by increasing the dose of glucocorticoids. Multivariate analyses were performed to identify patient, disease, or treatment factors associated with likelihood of complete response or overall improvement. A similar analysis was performed to identify covariates associated with time to treatment failure (defined as initiation of tertiary therapy or death not due to relapse of malignancy). Severe dysfunction in the skin, liver, and gut at the beginning of treatment was associated both with a decreased likelihood of complete response and an increased treatment failure rate. The times to treatment failure and the proportions of patients in various response categories were similar for primary and secondary treatment, suggesting that the potential efficacy of new immunosuppressive agents for treatment of acute GVHD can be assessed meaningfully in patients who have not responded adequately to initial therapy.
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PMID:A retrospective analysis of therapy for acute graft-versus-host disease: secondary treatment. 201 5

The therapeutic spectrum for ultraviolet radiation treatment of skin disease has continued to be broadened. Psoralen photochemotherapy is beneficial in chronic lichenoid graft-versus-host disease and disseminated granuloma annulare. This treatment is now being found more useful in atopic eczema and chronic photosensitivity with some modifications of the therapy. UV phototherapy has also been found useful in mild to moderate atopic eczema. The nature of these treatments is also changing with greater use of selective UV phototherapy and definition of the required schedule for maintenance treatment with UVB phototherapy. The mechanism of therapeutic benefit remains unknown although one possibility is selective phototoxicity for inflammatory cells in the dermis. Nonmelanoma skin cancer, premature aging of the skin and freckling are the main long-term adverse effects of these treatments.
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PMID:Recent advances in phototherapy and photochemotherapy of skin disease. 208 2

We have reviewed results of therapy in 740 patients with grades II-IV acute graft-versus-host disease (GVHD) after allogeneic marrow transplantation. At the beginning of therapy, 597 patients (81%) had rash, 369 (50%) had liver dysfunction and 396 (54%) had gut dysfunction. Initial treatment was with glucocorticoids (n = 531), cyclosporine (n = 170), antithymocyte globulin (ATG) (n = 156) or monoclonal antibody (n = 3) either singly (n = 633) or in combination (n = 107). Parameters of GVHD severity in each organ were recorded weekly, and evaluation of response was made using values at the initiation of secondary treatment or, for patients without such treatment, using values on day 29 of primary treatment or the last recorded value before death, whichever occurred first. Minimal criteria for improvement or progression were defined for each organ, but no attempt was made to define liver or gut outcome if another complication such as venocclusive disease or infectious enteritis was present. Improvement rates were 43% for skin disease, 35% for evaluable liver disease and 50% for evaluable gut disease. Overall complete or partial responses were seen in 44% of patients. Multivariate analyses were carried out to identify patient, disease or treatment factors associated with likelihood of overall improvement and likelihood of response in at least one organ. A similar analysis was also carried out to identify covariates associated with time to treatment failure (defined as initiation of secondary therapy or death not due to relapse of malignancy). In all three models, GVHD prophylaxis using cyclosporine combined with methotrexate was associated with favorable GVHD treatment outcome compared to prophylaxis with either agent alone, and treatment with glucocorticoids or cyclosporine was more successful than treatment with ATG. Other factors associated with unfavorable outcome in the model of time to treatment failure and also entered in one of the response models were recipient HLA disparity with the donor, presence of a liver complication other than GVHD, and early onset of GVHD. Results of this analysis indicate that glucocorticoids represent the best initial therapy available for treatment of acute GVHD, although much room for improvement remains.
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PMID:A retrospective analysis of therapy for acute graft-versus-host disease: initial treatment. 220 21

Because keratinocytes (KCs) express HLA-DR in a wide variety of skin diseases in which mononuclear leukocytes are observed in close apposition to KCs (i.e., graft-versus-host disease), and since gamma interferon (IFN-gamma) induces HLA-DR expression on KCs, we asked whether IFN-gamma treatment of KCs would influence the adherence of mononuclear leukocytes. When allogeneic peripheral blood mononuclear leukocytes (PBML) and a Leu-3+ T cell clone were coincubated with IFN-gamma-treated KCs (300 U/ml, 3 days), there was a marked increase in binding compared with nontreated KCs. Similar binding results were obtained using a cutaneous squamous carcinoma cell line (SCL-1) after IFN-gamma treatment. The IFN effect was relatively specific for IFN-gamma, as neither IFN-alpha nor -beta had any effect. Tumor necrosis factor exposure (500 U/ml, 3 days) increased the binding of the Leu-3+ T cell clone to both KCs and SCL-1 cells. Neutrophils displayed a less marked (but statistically significant) increase in binding to IFN-gamma-treated KCs. Using the Leu-3+ cell clone and SCL-1 cells, detailed kinetic analysis of the effect of IFN-gamma on binding was performed. The increased adherence between the cells began to appear after only 7 hours of treatment with r-IFN-gamma (300 U/ml) and reached a plateau at 48 hours, with significantly enhanced binding continuing for at least 48 hours after removal of IFN-gamma. The mechanism of binding was explored by preincubation of the PBML/Leu-3+ T cells with anti-LFA-1 (lymphocyte function-associated antigen) antibody (0.6-6.0 micrograms/ml), which totally inhibited the binding with no effect by anti-LFA-2 or -3 or class I or II antibodies despite documented binding of these antibodies to the cells. These results suggest that, after exposure to IFN-gamma, the ability of KCs to bind mononuclear leukocytes is strongly enhanced, and this adherence may be important in leukocyte trafficking in the skin as well as contributing to altered KC-leukocyte interaction, which may be of fundamental importance in a variety of skin disease.
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PMID:Recombinant gamma interferon increases the binding of peripheral blood mononuclear leukocytes and a Leu-3+ T lymphocyte clone to cultured keratinocytes and to a malignant cutaneous squamous carcinoma cell line that is blocked by antibody against the LFA-1 molecule. 244 90

Of the 31 cases of allogeneic bone marrow transplantation performed in the past 4 years at the National Taiwan University Hospital, 25 evaluable cases were retrospectively studied for incidence, severity, and risk factors of acute graft-versus-host disease (GVHD). The incidence was 60% (15/25) with the proportion of severe form (greater than or equal to grade II) being 53% (8/15). Skin involvement was the most common (13/15), gut the second (5/15), and liver the third (4/15). Most of the diagnosis of skin disease were confirmed by biopsy. Acute GVHD accounted for 3 deaths directly and was the next most common cause of death in post-transplant patients. The long-term survival rate for cases with grade 0-I disease was 65%, and that for those with grade II-III disease was 38%. Multivariate linear regression analysis of risk factors associated with severe form GVHD showed that the diagnosis of aplastic anemia was the most significant factor. Total lymphoid irradiation with or without plasmapheresis in aplastic anemia might contribute to the occurrence of severe acute GVHD.
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PMID:Acute graft-versus-host disease: a clinical report and analysis of risk factors. 280 59

Experimental models of chronic graft-versus-host disease have been advocated for studying the pathogenesis of scleroderma. However, microvascular abnormalities have not been documented in these models, whereas in man, cutaneous microvascular pathology (demonstrable by intravital microscopy) is a common feature of scleroderma. In our study scleroderma-like skin disease was evoked by allogeneic bone marrow transplantation in rats. Intravital microscopy of the skin of these animals showed strongly dilated capillaries, resembling the dilated loops in patients. This finding demonstrates that chronic graft-versus-host (scleroderma-like) disease may be pertinent to study the pathogenesis of microvascular injury in scleroderma.
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PMID:In vivo demonstration of microvascular pathology by intravital microscopy in experimental chronic graft-versus-host disease: analogy with scleroderma. 305 70

Sixteen patients with leukemia in relapse or second to third remission, 5 to 27 years old (median, 17), were given cyclophosphamide (60 mg/kg X 2) and total body irradiation (2.25 Gy for each of seven days) followed by unmodified marrow grafts from HLA-identical siblings. Patients did not receive posttransplant immunosuppression and were followed a median of nine months (range, 5-17). Prompt engraftment was sustained in 12 patients with a median time of 16 days (range, 10 to 63) to achieve 500 neutrophils/mm3. One patient failed to engraft, one had delayed engraftment, and two had late poor graft function. All 15 with engraftment developed moderate to life-threatening graft-v-host disease (GVHD, eight grade II and seven grade III-IV). This syndrome was hyperacute (median onset eight days [range, 7 to 29] posttransplant) and manifest by severe skin disease (14 patients at stage 3 and one at stage 4), fever (ten patients), and liver (four patients, stage 3-4) or gut (four patients, stage 3-4) involvement. Serial tissue biopsies confirmed acute GVHD in 13 of 15 patients. Ten were treated with antithymocyte globulin and cyclosporine (four survive), and four with corticosteroids (two survive). Actuarial survival to 17 months was 37%. Causes of death included interstitial pneumonia (four), infection (three), graft failure (one), venocclusive disease (one), and relapse of leukemia (one). Age-matched controls receiving standard methotrexate after transplant had comparable relapse-free survival but only a 25% incidence of grade II-IV acute GVHD (P less than .0001). We conclude that deleting posttransplant immunosuppression is associated with frequent and severe hyperacute GVHD, infectious complications, and occasional poor graft function.
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PMID:Hyperacute graft-v-host disease in patients not given immunosuppression after allogeneic marrow transplantation. 351 69

A 39-year-old woman with coexistent lichen planus, lichen sclerosus, and generalized morphea with ulcerations is described. Three additional cases were found in the files of the Mayo Clinic from 1950 to 1983, and these are summarized. Eight cases reviewed in the literature are also summarized. Coexistent lichenoid and sclerodermatous eruptions in graft-versus-host disease after bone marrow transplantation are noted as a model for this combined inflammatory and sclerotic dermatosis.
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PMID:Coexistence of lichen sclerosus, morphea, and lichen planus. Report of four cases and review of the literature. 400 88


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