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HEMATOPOIESIS Shwachman-Diamond Syndrome: An Inherited Preleukemic Bone Marrow Failure Disorder With Aberrant Hematopoietic Progenitors
"... Shwachman-Diamond syndrome (SD), an inherited disorder with varying cytopenias and a marked tendency for malig-nant myeloid transformation, is an important model for understanding genetic determinants in hematopoiesis. To define the basis for the faulty hematopoietic function, 13 patients with SD (2 ..."
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(2 of whom had myelodysplasia with a clonal cytogenetic abnormality) and 11 healthy marrow donors were studied. Patients with SD had significantly lower numbers of CD341 cells on bone marrow aspirates. SD CD341 cells plated directly in standard clonogenic assays showed markedly impaired colony
Durable Treatment-Free Remission after High-Dose Cyclophosphamide Therapy for Previously Untreated Severe Aplastic Anemia
"... Background: Severe aplastic anemia is a life-threatening bone marrow failure disorder. High-dose cyclophosphamide therapy fol-lowed by allogeneic bone marrow transplantation cures the dis-ease. However, it requires a suitable donor and carries the risk for graft-versus-host disease. A small pilot st ..."
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Background: Severe aplastic anemia is a life-threatening bone marrow failure disorder. High-dose cyclophosphamide therapy fol-lowed by allogeneic bone marrow transplantation cures the dis-ease. However, it requires a suitable donor and carries the risk for graft-versus-host disease. A small pilot
protein synthesis?
"... ABSTRACT The inherited bone marrow failure syndromes are clinically distinct but share some common features. Difficult to treat and typified by a poor prognosis, their pathogenesis is unknown. Recent findings that some patients with the erythroblastopenia Diamond-Blackfan anemia (DBA) have mutations ..."
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mutations in ribosomal proteins have led to the idea that this and perhaps other bone marrow failure disorders result from an inadequate supply of normally functioning ribosomes. According to this hypothesis, an insufficiency of the protein synthetic capacity limits the replicative potential of cells
Bone Marrow Failure Syndromes
, 2007
"... This review highlights some of the contributions that have appeared in the literature in the past decade on the pathogenesis and treatment of aplastic anemia (AA). This summary is brief because the field is vast, spaning from stem cell biology to stem cell disorders, from autoimmunity to transplanta ..."
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This review highlights some of the contributions that have appeared in the literature in the past decade on the pathogenesis and treatment of aplastic anemia (AA). This summary is brief because the field is vast, spaning from stem cell biology to stem cell disorders, from autoimmunity
Letter to the Editor DOI: 10.4274/Tjh.2013.0207 Lenalidomide-Induced Pure
, 2013
"... To the Editor, Pure red cell aplasia (PRCA) is a bone marrow failure disorder. Several drugs have been reported as having induced PRCA, but lenalidomide-induced PRCA is rarely reported. Here we present a patient with myelodysplastic syndrome (MDS) who developed PRCA after treatment with lenalidomide ..."
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To the Editor, Pure red cell aplasia (PRCA) is a bone marrow failure disorder. Several drugs have been reported as having induced PRCA, but lenalidomide-induced PRCA is rarely reported. Here we present a patient with myelodysplastic syndrome (MDS) who developed PRCA after treatment
Current insights into inherited bone marrow failure syndromes
, 2014
"... Inherited bone marrow failure syndrome (IBMFS) encompasses a heterogeneous and complex group of genetic disorders characterized by physical malformations, insufficient blood cell production, and increased risk of malignancies. They often have substantial phenotype overlap, and therefore, genotyping ..."
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Inherited bone marrow failure syndrome (IBMFS) encompasses a heterogeneous and complex group of genetic disorders characterized by physical malformations, insufficient blood cell production, and increased risk of malignancies. They often have substantial phenotype overlap, and therefore, genotyping
Bone Marrow Transplantation for Fanconi Anemia
, 1994
"... Fanconi anemia is a genetic disorder associated with diverse congenital abnormalities, progressive bone marrow failure, and increased risk of leukemia and other cancers. Affected persons often die before 30 years of age. Bone marrow trans-plantation is an effective treatment, but there are few data ..."
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Fanconi anemia is a genetic disorder associated with diverse congenital abnormalities, progressive bone marrow failure, and increased risk of leukemia and other cancers. Affected persons often die before 30 years of age. Bone marrow trans-plantation is an effective treatment, but there are few
Diagnosis, Genetics, and Management of Inherited Bone Marrow Failure Syndromes
"... The inherited bone marrow failure syndromes are traditionally considered to be pediatric disorders, but in fact, many of the patients now are diagnosed as adults, and many diagnosed as children now live to reach adulthood. The most common of these rare disorders include Fanconi anemia, dyskeratosis ..."
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The inherited bone marrow failure syndromes are traditionally considered to be pediatric disorders, but in fact, many of the patients now are diagnosed as adults, and many diagnosed as children now live to reach adulthood. The most common of these rare disorders include Fanconi anemia, dyskeratosis
Bone marrow failure, Cancer susceptibility
"... Improvement of genetic stability in lymphocytes from Fanconi anemia patients through the combined effect of α-lipoic acid and N-acetylcysteine bone marrow failure and early cancer development. ..."
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Improvement of genetic stability in lymphocytes from Fanconi anemia patients through the combined effect of α-lipoic acid and N-acetylcysteine bone marrow failure and early cancer development.
Bone Marrow Failure Syndromes: The Ribosomopathies
, 2012
"... This article was originally published in a journal published by OMICS Publishing Group, and the attached copy is provided by OMICS Publishing Group for the author’s benefit and for the benefit of the author’s institution, for commercial/research/educational use including without limitation use in in ..."
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This article was originally published in a journal published by OMICS Publishing Group, and the attached copy is provided by OMICS Publishing Group for the author’s benefit and for the benefit of the author’s institution, for commercial/research/educational use including without limitation use in instruction at your institution, sending it to specific colleagues that you know, and providing a copy to your institution’s administrator. All other uses, reproduction and distribution, including without limitation commercial reprints, selling or licensing copies or access, or posting on open internet sites, your personal or institution’s website or repository, are requested to cite properly.
Results 1 - 10
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1,670