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)

Bone marrow transplantation (BMT) has been increasingly applied to a variety of potentially fatal diseases in childhood. However, trends of indication of BMT are changing because chemotherapy in leukemia and immunosuppressive therapy with/without colony stimulating factor in aplastic anemia are improving. Several progresses have been noted in matched unrelated BMT and peripheral blood stem cell transplantation as well as in sibling BMT or autologous BMT. Many efforts are being made to decrease rejection rate or leukemia relapse and to improve quality of life by new conditioning regimens. Attempts to induce GVL effects or syngeneic GVHD are currently under progress. The quality of life in long term surviving children are generally good and acceptable, although delay in growth, infertility, cataract and obstructive lung disease are seen in a few patients.
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PMID:[Current problems in pediatric bone marrow transplantation]. 831 25

For the last 40 years it has been considered that during pregnancy fetal antigens are expressed in a different manner in comparison with other grafts. Current data indicates that the acceptance of the fetus by the mother depends on the lack of expression of the polymorphic antigens and the production of hormones that act as immunological suppressors. During pregnancy there exists immunological recognition of the trophoblast antigens, but these antigens are not polymorphic and thus do not permit the identification of cytolytic T and natural killers cells. This structure also produces hormones that contribute to the diminished production and proliferation of T cells. It has been demonstrated that there exists an increase in the production by the trophoblast of complement inhibitors (DAF, CD46) and in the secretion of hormones such as progesterone, alpha-fetoprotein, steroids and prostanglandins. The identification of these immunological factors and mechanisms may be fundamental in the search for treatment regimens for such illnesses as cancer, infertility, spontaneous abortions, transplant rejection and graft versus host disease.
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PMID:[Immunology of the fetal-maternal relationship]. 890 Oct 36

A case of disseminated superficial porokeratosis (DSP) is reported in a black man 5 years after autologous bone marrow transplantation (BMT) for acute promyelocytic leukemia. Porokeratosis is a rare hyperkeratotic disorder arising from clonal keratinocytes with a high potential to develop squamous cell carcinoma. Inherited forms are classical but recent observations of acquired porokeratosis have been reported in immunocompromized patients (AIDS, immune disorders, immune suppressive drugs or organ transplantation). Two cases of DSP have been reported after allogeneic BMT in patients treated for chronic GVHD. Our case is the first one after autologous BMT, in a black man, on no immunosuppressive drug at the time of diagnosis of DSP. Hematopoietic and immune reconstitution was apparently complete. The cancer-prone character of porokeratosis could be favored by total body irradiation used in conditioning regimen. Thus, porokeratosis has to be associated with other late effects after BMT such as HCV seropositivity, cataract and infertility that were observed in this patient.
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PMID:Disseminated superficial porokeratosis after autologous bone marrow transplantation. 901 35

Close follow-up care for the patient who has received either a bone marrow or peripheral blood stem cell transplantation is essential for the first year after the transplantation. The patient can experience many side effects, such as compromised immune system, graft versus host disease, veno-occlusive disease, and infertility. The role of the clinic or homecare nurse is to provide thorough assessment so as to detect treatable problems early and to identify nontreatable problems that might be affecting the patient's quality of life.
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PMID:Ambulatory care after bone marrow or peripheral blood stem cell transplantation. 941 Jun 46

To evaluate the late-effects of allogeneic bone marrow transplantation (BMT) on endocrine function 20 adults (10 females, 10 males) with hematological malignancies were studied after a mean of 3.2 years (range 1.0-10.0) following BMT. The mean age of patients at the time of BMT was 39 years. Dynamic tests of the hypothalamic-pituitary axis included growth hormone releasing hormone (GHRH), gonadotropin releasing hormone (GnRH) and thyrotropin releasing hormone (TRH) stimulations with measurements of serum growth hormone (GH), follicle stimulating hormone (FSH), luteinizing hormone (LH), thyrotropin (TSH) and prolactin (PRL) responses. Adrenal function was assessed with the adrenocorticotropin (ACTH) test. Five patients (25%) had a subnormal GH response to GHRH stimulation, but all had a normal serum insulin-like growth factor I (IGF-I) value. There was an inverse nonlinear relationship between the body mass index (BMI; kg/m2) and GH response but no relation between the GH response and total body irradiation (TBI), intrathecal treatment or occurrence of graft-versus-host disease. In females, serum FSH and LH basal levels and responses to GnRH, in spite of oestrogen substitution therapy in 9/10 patients, indicated ovarian failure and early menopause. Most responses to GnRH were delayed. All males had elevated serum basal FSH levels indicating damage in seminiferous tubulus and infertility. Serum basal LH was elevated only in four males but testosterone values were all within normal limits. However, the mean free androgen index (FAI) was in the low normal range, and two subjects had abnormally low FAI. Serum free thyroxine (fT4) levels were normal in all but one, but an exaggerated TSH response to TRH occurred in seven patients (35%). Four of them had received TBI and one total nodal irradiation suggesting radiation-induced damage to the thyroid gland. In 19 of the 20 patients, adrenal function judged with ACTH test was normal. We conclude that functional impairments of the hypothalamus-pituitary-gonad/thyroid axis are common while disturbances in GH, adrenal and prolactin occur less often in patients after intensive treatment and BMT. Typically, the target organ is more commonly affected than the hypothalamus-pituitary axis. In spite of normal serum testosterone and LH values, serum FAI may reveal androgen deficiency.
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PMID:Long-term effects of allogeneic bone marrow transplantation (BMT) on pituitary, gonad, thyroid and adrenal function in adults. 972 67

Search for an etiology of bronchiectasis consists in identifying constitutional or acquired defense mechanisms of the respiratory mucosa. The question is timely because causes change. In developing countries, presumed sequelae of infection account for about 30% of the cases despite vaccination campaigns, control of endemic tuberculosis, and widespread use of antibiotics. Genetic diseases account for 20% of the causes when identified by high-performance prospective diagnostic tests (CFTR mutation). Computed tomography enables the identification of frequent associations between bronchiectasis and rheumatoid disease or ulcerative colitis. Recent diseases such as HIV infection or GVHD can also lead to bronchiectasis. Nevertheless, the cause remains unknown in 30-50% of patients. After a detailed analysis of the clinical presentation and diagnostic criteria specific for each etiology, we propose a two-phase diagnostic procedure. The first step, used for all patients (careful history taking, physical examination, imaging, bronchofibroscopy, limited blood tests) enables detecting localized bronchial obstacles and obvious etiologies (situs inversus of primary ciliary dyskinesia, known systemic disease, HIV...). If the first step is negative, the second phase is oriented by the clinical context. Sequelae of infection (tuberculosis...) in older subjects or migrants, a genetic cause in younger subjects, particularly if there is a familial history and/or infertility, a systemic disease or allergic bronchopulmonary aspergillosis if there is an extra-respiratory context. This etiological search should help improve patient management and provide a better prognosis and prevention of bronchiectasis.
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PMID:[Etiological work-up for bronchectasia in adults]. 1568 8

A total of 52 children, age 9 or over and at least 3 years (median=8) beyond SCT for leukaemia (n=32) or nonmalignant diseases, participated in a single-centre study of health and quality of life (QoL). QoL and self-esteem were assessed with SCHQ-CF87, a generic multidimensional self-report instrument, and with 'I think I am'. As a group, the children had good QoL, but were below norm in the bodily pain (P<0.05), general health and self-esteem dimensions (P<0.01). Lansky or Karnofsky function levels were at a median of 90. Sense of coherence (SOC-13) was normal and correlated with SCHQ-CF87. Most children were subjectively and objectively in good health according to a self-assessment symptom inventory or by a medical record-based scoring of late effects, although pain was commonly reported. A total of 25% of the patients were rated as having moderate to severe late effects, without considering cataracts or infertility. Neither age at SCT, gender, malignant vs nonmalignant disease, nor stature influenced QoL significantly. Children with moderate to severe chronic graft-versus-host disease or cognitive deficits had lower QoL in some dimensions. No correlation was, however, found between the physician-rated total late effects score and overall QoL. Contrarily, QoL was clearly related to the degree of self-rated symptoms.
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PMID:Quality of life and health in children following allogeneic SCT. 1593 8

The development of reduced-intensity conditioning (RIC) and the success of BMT for paediatric sickle cell disease (SCD) have raised the possibility of revisiting this prospect in adults as well. In a chronic debilitating disorder managed with supportive therapy, the patients' perception is critical in the advancement of any potential curative therapy. To explore this aspect, we undertook a questionnaire-based survey on 30 adults with SCD. Sixty two per cent of the patients were ready to accept a transplant-related mortality (TRM) >10%; 30% of them a TRM >30%. A risk of graft failure (GF) >10% was acceptable to 64%, with a risk >30% acceptable to 41%. Infertility was acceptable to only 50%. Chronic graft-versus-host disease (GVHD) was unacceptable to the majority (80%). Seventy six per cent% of patients had a full sibling and 60% were willing to participate in a clinical trial of RIC transplantation. This survey suggests that the majority of adults with SCD might be willing to consider a curative option such as RIC transplantation even with a high TRM or GF. The major concerns relate to chronic GVHD and infertility. There is an urgent need to explore RIC transplants in SCD patients within the framework of a clinical trial, considering patient perception regarding cure and complications.
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PMID:A survey on patient perception of reduced-intensity transplantation in adults with sickle cell disease. 1733 83

Chronic myeloid leukemia (CML) is a relatively rare hematopoietic malignancy in the pediatric and adolescent population. This makes it difficult to perform clinic trials that can define the best therapeutic option when considering the impact of tyrosine kinase inhibitors (TKIs) versus the established approach of allogeneic hematopoietic cell transplantation (HCT). With the relatively low toxicity of TKIs, there are little data regarding when HCT or long-term TKI therapy is a better option. There are even less data regarding the duration of TKI treatment in the pediatric CML in chronic phase (CML-CP) patients who may receive over 60 years of therapy. As children and adolescent are treated for longer times with TKIs, it has become clear that toxicities may make long-term TKI therapy less attractive compared to allogeneic HCT. HCT has the long-term complications of growth failure, infertility, chronic graft-versus-host disease (GVHD), metabolic syndrome, and secondary malignancies, whereas prolonged TKIs may cause growth failure, hepatic, and cardiac complications. Moreover, HCT is a potentially curative intervention, whereas TKI is not curative, requiring prolonged exposure. In this article, we discuss the relative merit of the 2 therapeutic approaches and recommend that all children and adolescents with CML-CP should initially be treated with imatinib and maintained with TKI therapy indefinitely if there is a good response. We recommend that allogeneic HCT with an HLA-identical sibling donor or closely matched unrelated donor be considered for patients with treatment failure or recurrence after receiving salvage second-generation TKI treatment. We also conclude that randomized international trials are urgently needed to evaluate the best therapies for pediatric CML.
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PMID:Controversies in the treatment of CML in children and adolescents: TKIs versus BMT? 2119

Female long-term survivors of allogeneic hematopoietic stem cell transplantation (allo-HSCT) incur a significant burden of late effects. Genital graft-versus-host disease (GVHD), human papillomavirus (HPV) reactivation, ovarian failure and infertility, sexual dysfunction, and osteoporosis are concerns that can significantly impact quality of life. This review examines the risk, pathogenesis, clinical presentation, and implications of these common complications. Recommendations are provided for evaluation and management of these late effects and other obstetric and gynecologic issues that may arise in this patient population.
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PMID:Female long-term survivors after allogeneic hematopoietic stem cell transplantation: evaluation and management. 2222 88


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