Fanconi's anemia: Current concepts

Blanche P. Alter

Research output: Contribution to journalArticle

Abstract

Fanconi's anemia is an autosomal recessive disorder with a high incidence (>90%) of aplastic anemia and a premalignant component with a >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.

Original languageEnglish (US)
Pages (from-to)170-176
Number of pages7
JournalJournal of Pediatric Hematology/Oncology
Volume14
Issue number2
StatePublished - 1992
Externally publishedYes

Keywords

  • Aplastic anemia
  • Bone marrow
  • Fanconi's anemia
  • Leukemia
  • Pregnancy
  • Preleukemia

ASJC Scopus subject areas

  • Hematology
  • Oncology
  • Pediatrics, Perinatology, and Child Health

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