Document Detail

Fanconi's anemia. Current concepts.
MedLine Citation:
PMID:  1530123     Owner:  NLM     Status:  MEDLINE    
Fanconi's anemia is an autosomal recessive disorder with a high incidence (greater than 90%) of aplastic anemia and a premalignant component with a greater than 10% risk of leukemia or solid tumors. The diagnosis of Fanconi's anemia depends on increased chromosomal breakage in lymphocytes following treatment with a DNA cross-linking agent; patients have been identified who are clinically well and whose physical appearance is normal. Although bone marrow or cord blood transplants can be curative, treatment for the aplastic anemia usually depends on androgens. Close to 20 patients with Fanconi's anemia have delivered normal babies, and the mothers' hematologic status was not significantly adversely affected by the pregnancy. A few patients have clonal cytogenetic abnormalities in their bone marrow that do not necessarily indicate leukemic transformation, but further follow-up is important. Studies of in vitro erythropoiesis indicate a correlation between the clinical hematologic status and the presence of erythroid progenitors in the blood or bone marrow. Certain hematopoietic growth factors do increase growth in vitro, suggesting that new types of therapy may become available. Not every patient has a poor prognosis. There are now many adults with Fanconi's anemia, some with families of their own.
B P Alter
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Publication Detail:
Type:  Journal Article; Research Support, Non-U.S. Gov't; Review    
Journal Detail:
Title:  The American journal of pediatric hematology/oncology     Volume:  14     ISSN:  0192-8562     ISO Abbreviation:  Am J Pediatr Hematol Oncol     Publication Date:  1992 May 
Date Detail:
Created Date:  1992-10-20     Completed Date:  1992-10-20     Revised Date:  2006-11-15    
Medline Journal Info:
Nlm Unique ID:  7908071     Medline TA:  Am J Pediatr Hematol Oncol     Country:  UNITED STATES    
Other Details:
Languages:  eng     Pagination:  170-6     Citation Subset:  IM    
Polly Annenberg Levee Hematology Center, Department of Medicine, Mount Sinai School of Medicine, New York, NY 10029.
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MeSH Terms
Androgens / therapeutic use
Bone Marrow Transplantation
Child, Preschool
Fanconi Anemia* / diagnosis,  genetics,  mortality,  physiopathology,  therapy
Genes, Recessive
Infant, Newborn
Life Tables
Middle Aged
Pregnancy Complications, Hematologic
Preleukemia / genetics
Survival Analysis
Reg. No./Substance:

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