Blood. 1989 Apr;73(5):1168-74.

Diamond-Blackfan anemia: promotion of marrow erythropoiesis in vitro by recombinant interleukin-3.

Blood

D S Halperin, Z Estrov, M H Freedman

PMID: 2649168

Abstract

To clarify the defective erythropoiesis in eight patients with Diamond-Blackfan anemia, we studied their bone marrow response in vitro to recombinant human interleukin-3 (IL-3) and recombinant granulocyte-macrophage colony-stimulating factor (GM-CSF). In an erythropoietin-containing assay system, specimens from six of the eight patients yielded low numbers of erythroid colonies compared to control values, and in five of these no erythropoietin dose-response could be elicited. Addition of IL-3, GM-CSF or both to cultures from the six patients had no effect on CFU-E-derived colonies. In contrast, IL-3 but not GM-CSF induced a marked increase in the number (183%) and size of the BFU-E-derived colonies in five of the six cases and partially corrected the impaired dose-response to erythropoietin in four. Bone marrow from the other two patients yielded numbers of CFU-E and BFU-E colonies comparable to controls and manifested similar increments in colonies with increasing concentrations of erythropoietin. When IL-3 was added to these cultures, further increments were observed in the number and size of BFU-E colonies. We conclude that IL-3 enhanced the marrow erythropoiesis in most of the patients and exerted a corrective effect on the aberrant colony formation in the presence of erythropoietin. The data raise the possibility of IL-3 as a therapeutic agent in Diamond-Blackfan anemia.

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Substances

• Colony-Stimulating Factors
• Growth Substances
• Interleukin-3
• Recombinant Proteins
• Granulocyte-Macrophage Colony-Stimulating Factor

MeSH terms

• Adolescent
• Adult
• Anemia, Aplastic / blood
• Anemia, Aplastic / pathology
• Bone Marrow / pathology
• Child
• Child, Preschool
• Colony-Forming Units Assay
• Colony-Stimulating Factors / pharmacology
• Erythropoiesis / drug effects
• Female
• Granulocyte-Macrophage Colony-Stimulating Factor
• Growth Substances / pharmacology
• Hematopoietic Stem Cells / pathology
• Hematopoietic Stem Cells / physiology
• Humans
• Infant
• Interleukin-3 / pharmacology
• Male
• Recombinant Proteins / pharmacology

Publication Types

• Journal Article
• Research Support, Non-U.S. Gov't

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