Gene/Protein Disease Symptom Drug Enzyme Compound
Pivot Concepts:   Target Concepts:
Query: UMLS:C0002874 (aplastic anemia)
 5,905 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Bone marrow transplantation in childhood is an established treatment modality for aplastic anemia, the acute and chronic leukemias, and severe combined immune deficiency. Recently, experience with this treatment has also been favorable with small numbers of children who have Wiskott-Aldrich syndrome, several types of inherited storage diseases, Fanconi's anemia, thalassemia, infantile malignant osteopetrosis, and selected cases of lymphoma and other solid tumors. The psychosocial impact and financial costs of bone marrow transplantation can be substantial. Multi-institutional, prospective, randomized trials that would compare transplantation and conventional therapy are necessary to establish the indications and precise timing for this procedure. Further development of monoclonal antibodies, a better understanding of the histocompatibility antigen systems, and improvement in pretransplantation conditioning regimens should increase the spectrum of effectiveness for bone marrow transplantation in the coming years.
 ...
PMID:Bone marrow transplantation for diseases of childhood. 636 12

Immune-mediated inhibition of hematopoiesis has been suspected as a major cause of the suppressed growth of progenitor cells in aplastic anemia (AA). Overproduction of TNF-alpha by bone marrow and peripheral blood-derived cells was shown to be of pathogenetic importance. Genetic factors have been suggested by higher specific histocompatibility antigen frequencies among AA patients as a group and among those unresponsive to immunosuppressive therapy with cyclosporine. In the present work we expand on the evidence for the contribution of the TNF system to therapeutic responses in patients with AA. The response to immunosuppressive therapy at 3 months was found to be significantly more frequent among carriers of the TNF2 (TNF -308 A) gene (TNF2 homozygotes and heterozygotes of the TNF-alpha promoter/enhancer polymorphism) than among those patients not carrying the TNF2 gene. The allelic distribution of the LT-alpha (TNF-beta) NcoI and IL-1 receptor antagonist variable number tandem repeat (VNTR) polymorphisms did not differ among the subgroups of patients. The association of the TNF-alpha genotype with a response to immunosuppressive therapy points to an immunogenetic association that may contribute to the pathogenesis and therapeutic response of aplastic anemia.
 ...
PMID:Clinical relevance of the TNF-alpha promoter/enhancer polymorphism in patients with aplastic anemia. 1242 37