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Variable in vitro erythropoiesis in patients with transient erythroblastopenia of childhood.

Transient erythroblastopenia of childhood (TEC) is a pure red cell aplasia which primarily affects children in the infant and toddler age group. The clinical syndrome of TEC is well defined and is characterized by moderate to severe anemia with reticulocytopenia, selective aplasia of the erythroid b...

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Detalles Bibliográficos
Autores principales: Ritchey, A. K., Dainiak, N., Hoffman, R.
Formato: Texto
Lenguaje:English
Publicado: Yale Journal of Biology and Medicine 1985
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC2589835/
https://www.ncbi.nlm.nih.gov/pubmed/4013369
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author Ritchey, A. K.
Dainiak, N.
Hoffman, R.
author_facet Ritchey, A. K.
Dainiak, N.
Hoffman, R.
author_sort Ritchey, A. K.
collection PubMed
description Transient erythroblastopenia of childhood (TEC) is a pure red cell aplasia which primarily affects children in the infant and toddler age group. The clinical syndrome of TEC is well defined and is characterized by moderate to severe anemia with reticulocytopenia, selective aplasia of the erythroid bone marrow elements, and spontaneous recovery, usually within a month of presentation. We utilized the plasma clot tissue culture technique to explore the defect of erythropoiesis in seven patients with TEC. Culture of bone marrow at diagnosis in four patients revealed an increased erythroid proliferative capacity in one and a decreased capacity in three. The former patient plus three additional patients were found to have a transient serum inhibitor of erythroid colony formation in autologous and allogeneic systems. The three patients with diminished erythroid proliferative capacity had no demonstrable serum inhibitor, and in one patient studied the erythroid proliferative capacity became supernormal after recovery. We conclude that although TEC has a characteristic clinical picture, in vitro studies reveal a variable expression of the erythropoietic defect and support the hypothesis of a heterogeneous pathogenesis of this disorder.
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spelling pubmed-25898352008-11-28 Variable in vitro erythropoiesis in patients with transient erythroblastopenia of childhood. Ritchey, A. K. Dainiak, N. Hoffman, R. Yale J Biol Med Research Article Transient erythroblastopenia of childhood (TEC) is a pure red cell aplasia which primarily affects children in the infant and toddler age group. The clinical syndrome of TEC is well defined and is characterized by moderate to severe anemia with reticulocytopenia, selective aplasia of the erythroid bone marrow elements, and spontaneous recovery, usually within a month of presentation. We utilized the plasma clot tissue culture technique to explore the defect of erythropoiesis in seven patients with TEC. Culture of bone marrow at diagnosis in four patients revealed an increased erythroid proliferative capacity in one and a decreased capacity in three. The former patient plus three additional patients were found to have a transient serum inhibitor of erythroid colony formation in autologous and allogeneic systems. The three patients with diminished erythroid proliferative capacity had no demonstrable serum inhibitor, and in one patient studied the erythroid proliferative capacity became supernormal after recovery. We conclude that although TEC has a characteristic clinical picture, in vitro studies reveal a variable expression of the erythropoietic defect and support the hypothesis of a heterogeneous pathogenesis of this disorder. Yale Journal of Biology and Medicine 1985 /pmc/articles/PMC2589835/ /pubmed/4013369 Text en
spellingShingle Research Article
Ritchey, A. K.
Dainiak, N.
Hoffman, R.
Variable in vitro erythropoiesis in patients with transient erythroblastopenia of childhood.
title Variable in vitro erythropoiesis in patients with transient erythroblastopenia of childhood.
title_full Variable in vitro erythropoiesis in patients with transient erythroblastopenia of childhood.
title_fullStr Variable in vitro erythropoiesis in patients with transient erythroblastopenia of childhood.
title_full_unstemmed Variable in vitro erythropoiesis in patients with transient erythroblastopenia of childhood.
title_short Variable in vitro erythropoiesis in patients with transient erythroblastopenia of childhood.
title_sort variable in vitro erythropoiesis in patients with transient erythroblastopenia of childhood.
topic Research Article
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC2589835/
https://www.ncbi.nlm.nih.gov/pubmed/4013369
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