| Thrombocytopenia with absent radii syndrome: studies on serum thrombopoietin levels and megakaryopoiesis in vitro. | |
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MedLine Citation:
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PMID: 9482417 Owner: NLM Status: MEDLINE |
Abstract/OtherAbstract:
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PURPOSE: The pathogenesis of thrombocytopenia in patients with thrombocytopenia with absent radii (TAR) syndrome has not been clarified yet. PATIENTS AND METHODS: This is the first report of a Japanese patient with TAR syndrome. We studied his megakaryopoiesis in vitro and serum levels of thrombopoietin (TPO). RESULTS: Serum levels of TPO in the patient with TAR syndrome were comparable with those of an age-matched control. The bone marrow cells from the patient with TAR syndrome actually generated megakaryocyte colonies in the presence of TPO and the numbers were significantly greater than those from the age-matched control marrow. However, megakaryocyte colonies from the marrow cells with TAR syndrome contained a much lower number of cells per colony and the size of the individual megakaryocytes appeared to be smaller. CONCLUSION: These data suggest that megakaryocyte progenitors from patients with TAR syndrome may have decreased proliferative and differentiative capacity to respond to TPO, leading to thrombocytopenia. |
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Authors:
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I Sekine; T Hagiwara; H Miyazaki; K Hirayama; H Dobashi; K Kogawa; S Yoshioka |
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Publication Detail:
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Type: Case Reports; Journal Article |
Journal Detail:
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Title: Journal of pediatric hematology/oncology Volume: 20 ISSN: 1077-4114 ISO Abbreviation: J. Pediatr. Hematol. Oncol. Publication Date: 1998 Jan-Feb |
Date Detail:
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Created Date: 1998-03-05 Completed Date: 1998-03-05 Revised Date: 2011-10-06 |
Medline Journal Info:
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Nlm Unique ID: 9505928 Medline TA: J Pediatr Hematol Oncol Country: UNITED STATES |
Other Details:
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Languages: eng Pagination: 74-8 Citation Subset: IM |
Affiliation:
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Department of Pediatrics, National Defense Medical College, Tokorozawa, Saitama, Japan. |
Export Citation:
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| MeSH Terms | |
Descriptor/Qualifier:
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Hematopoiesis* Humans Infant, Newborn Male Megakaryocytes / physiology* Syndrome Thrombocytopenia / blood* Thrombopoietin / blood* |
| Chemical | |
Reg. No./Substance:
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9014-42-0/Thrombopoietin |
From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine
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