In vitro growth and regulation of bone marrow enriched CD34+ hematopoietic progenitors in Diamond-Blackfan anemia

G. P. Bagnara, G. Zauli, L. Vitale, P. Rosito, V. Vecchi, G. Paolucci, G. C. Avanzi, U. Ramenghi, F. Timeus, V. Gabutti

Research output: Contribution to journalArticle

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Abstract

Diamond-Blackfan anemia (DBA) is a congenital red blood cell aplasia. No clear explanation has been given of its defective erythropoiesis, although different humoral or cellular inhibitory factors have been proposed. To clarify the nature of this defect we studied the effect of several human recombinant growth factors on an enriched CD34+ population obtained from the bone marrow of 10 DBA patients. We observed a defect underlying the early erythroid progenitors, which were unresponsive to several growth factors (erythropoietin, interleukin-3 [IL-3], IL-6, granulocyte-macrophage colony-stimulating factor [GM-CSF], erythroid potentiating activity), either alone or in association. The production of cytokines was not impaired, and high levels of IL-3 and GM-CSF were found in phytohemagglutinin-leukocyte-conditioned medium (PHA-LCM) when tested with a sensitive biologic assay on the M-07E cell line. Hematopoietic stem cells in DBA patients may be induced to differentiate to the granulocyte megakaryocyte, but not the erythroid compartment, as shown after CD34+ cell preincubation with IL-3. Addition of the stem cell factor to IL-3 and erythropoietin induces a dramatic in vitro increase in both the number and the size of BFU-E, which also display a normal morphologic terminal differentiation.

Original languageEnglish
Pages (from-to)2203-2210
Number of pages8
JournalBlood
Volume78
Issue number9
Publication statusPublished - Nov 1 1991

Fingerprint

Diamond-Blackfan Anemia
Diamond
Interleukin-3
Bone
Bone Marrow
Granulocyte-Macrophage Colony-Stimulating Factor
Growth
Erythropoietin
Intercellular Signaling Peptides and Proteins
Cells
Defects
Erythroid Precursor Cells
Stem Cell Factor
Megakaryocytes
Erythropoiesis
Phytohemagglutinins
Conditioned Culture Medium
Hematopoietic Stem Cells
Stem cells
Granulocytes

ASJC Scopus subject areas

  • Hematology

Cite this

Bagnara, G. P., Zauli, G., Vitale, L., Rosito, P., Vecchi, V., Paolucci, G., ... Gabutti, V. (1991). In vitro growth and regulation of bone marrow enriched CD34+ hematopoietic progenitors in Diamond-Blackfan anemia. Blood, 78(9), 2203-2210.

In vitro growth and regulation of bone marrow enriched CD34+ hematopoietic progenitors in Diamond-Blackfan anemia. / Bagnara, G. P.; Zauli, G.; Vitale, L.; Rosito, P.; Vecchi, V.; Paolucci, G.; Avanzi, G. C.; Ramenghi, U.; Timeus, F.; Gabutti, V.

In: Blood, Vol. 78, No. 9, 01.11.1991, p. 2203-2210.

Research output: Contribution to journalArticle

Bagnara, GP, Zauli, G, Vitale, L, Rosito, P, Vecchi, V, Paolucci, G, Avanzi, GC, Ramenghi, U, Timeus, F & Gabutti, V 1991, 'In vitro growth and regulation of bone marrow enriched CD34+ hematopoietic progenitors in Diamond-Blackfan anemia', Blood, vol. 78, no. 9, pp. 2203-2210.
Bagnara GP, Zauli G, Vitale L, Rosito P, Vecchi V, Paolucci G et al. In vitro growth and regulation of bone marrow enriched CD34+ hematopoietic progenitors in Diamond-Blackfan anemia. Blood. 1991 Nov 1;78(9):2203-2210.
Bagnara, G. P. ; Zauli, G. ; Vitale, L. ; Rosito, P. ; Vecchi, V. ; Paolucci, G. ; Avanzi, G. C. ; Ramenghi, U. ; Timeus, F. ; Gabutti, V. / In vitro growth and regulation of bone marrow enriched CD34+ hematopoietic progenitors in Diamond-Blackfan anemia. In: Blood. 1991 ; Vol. 78, No. 9. pp. 2203-2210.
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