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Erythropoiesis in Fanconi's anemia
BP Alter, ME Knobloch and RS Weinberg
Department of Medicine, Polly Annenberg Levee Hematology Center, Mount
Sinai School of Medicine, New York, NY 10029.
Fanconi's anemia (FA) is an autosomal recessive condition in which greater
than 90% of the homozygotes develop aplastic anemia. To determine the
relation between erythroid progenitors and clinical status, blood and
marrow mononuclear cells were cultured in methyl cellulose with
erythropoietin, plus other hematopoietic growth factors, and growth in
normal oxygen (20%) was compared with growth in low, physiologic oxygen
(5%). Peripheral blood cultures were performed from 24 patients, and
marrows from six. Patients were classified into six clinical groups. Group
1: Severe aplasia, transfused; one patient; no erythroid progenitors. Group
2: Severe, transfused, androgen unresponsive; one patient; no blood
burst-forming units-erythroid (BFU- E). Group 3: Androgen responsive; eight
patients, with decreased blood BFU-E. Group 4: Aplastic, about to start
treatment; two patients; below normal numbers of colony-forming
units-erythroid (CFU-E) and BFU-E. Group 5: Stable, with mild anemia,
and/or thrombocytopenia, and/or macrocytosis; seven patients; with below
normal numbers of blood BFU-E. Group 6: Hematologically normal; five
patients; blood BFU-E low normal to normal. One marrow had normal numbers
of CFU-E and BFU-E. Incubation in 5% oxygen doubled CFU-E and BFU-E only in
the patients with close to normal or normal growth in 20% oxygen. Hemin and
interleukin-3 increased growth slightly in those cultures where there was
some growth with erythropoietin alone. Our data show that there is a
correlation between current clinical status and in vitro erythropoiesis.
Cultures of erythroid progenitors may also be useful predictors of
hematologic prognosis in FA, although our follow-up period is too short to
prove this hypothesis.
Volume 78,
Issue 3,
pp. 602-608,
08/01/1991
Copyright © 1991 by The American Society of Hematology
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