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)

In the past two decades there have been a number of advances in the treatment of chronic myeloid leukemia (CML) in chronic phase. There has been very little progress in the management of advanced disease. Hydroxyurea and interferon-alpha have replaced busulfan as first-line therapy in chronic phase. Hydroxyurea provides excellent control of the disease for periods of months or years and has little toxicity. Interferon-alpha induces a reduction in the Philadelphia chromosome in a substantial minority of patients. Interferon-alpha may also prolong survival. Neither hydroxyurea nor interferon-alpha is able to cure the disease. Patients in chronic phase may be cured by allogeneic bone marrow transplantation (BMT). This procedure has a number of limitations, however, including limited availability, high cost, and substantial morbidity and mortality. Current challenges in BMT are to increase its applicability, to reduce graft-versus-host disease, and to augment graft-versus-leukemia effects. The role of autologous transplantation in CML remains speculative.
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PMID:Approaches to the treatment of chronic myeloid leukemia. 791 35

The management of chronic myeloid leukemia (CML) patients who relapse after allogeneic bone marrow transplantation (BMT) is difficult. Hydroxyurea, alpha interferon, second BMT and leukocytes infusion are various options but none of these approaches is clearly optimal. Hydroxyurea controls the symptoms in most patients without any apparent survival benefit. Alpha interferon (IFN) results in haematological remission in most cases with partial or total Philadelphia negativity in 20-30% of patients. Whether IFN therapy prolongs survival is not yet certain. Second BMT results in successful outcome in about half of the patients, however toxicity to the preparatory regimen, post transplant venocclusive disease and acute graft versus host disease are all major complications. An interval of less than 6 months between the initial and second BMT is generally associated with a poor outcome. Buffy coat infusions from the original donor have resulted in a cytogenetic remission in most patients. Less intensive preparatory regimes, donor buffy coat infusion and the use of biological response modifiers post transplant in order to augment the graft versus leukemia effect in high risk patients may indeed be possible areas of improvement in future studies.
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PMID:Chronic myeloid leukemia: management of relapse after allogeneic bone marrow transplantation. 822 Jan 14

Sickle-cell disease is a hereditary haemoglobinopathy caused by a mutation in the beta-globin gene. The disease is characterised by recurrent vaso-occlusive crises resulting in severe organ damage and a sharply reduced life expectancy. The formation of haemoglobin-S polymers in hypoxic conditions plays a pivotal role in sickle-cell disease and produces the characteristic phenotype of sickle-shaped erythrocytes that promote vasoocclusion. Endothelial cell activation, enhanced erythrocyte and leukocyte adhesion, vasoconstriction and coagulation activation play an important role in vaso-occlusive crises. Treatment of pain and hydration remain the main interventions in the management ofvaso-occlusive crises. Hydroxyurea has been shown to prevent vaso-occlusive crises by increasing the amount of foetal haemoglobin. Allogeneic stem-cell transplantation is the only curative therapy. However, transplantation-related mortality, graft-versus-host disease and the limited availability of HLA-identical donors restrict this therapeutic option.
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PMID:[Pathophysiology and treatment of sickle-cell disease]. 1653 53