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Ataluren ‐driven Restoration of Shwachman‐Bodian‐Diamond Syndrome Protein Function in Shwachman‐Diamond Syndrome Bone Marrow Cells

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Source: American Journal of Hematology - Category: Hematology Authors: Tags: Research Article Source Type: research

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AbstractPrimary graft failure can be a cause of early morbidity and mortality after allogeneic hematopoietic stem cell transplantation (HSCT), as it leads to a high risk of severe infections and bleeding. Splenomegaly is associated with primary graft failure in patients of myelofibrosis, but the association between splenomegaly and outcomes after HSCT in patients with myeloid malignancies has not been previously evaluated. The aim of this study was to investigate the effect of spleen volume on engraftment kinetics in patients with acute myeloid leukemia (AML) and myelodysplastic syndrome (MDS). We enrolled 85 patients. The...
Source: Annals of Hematology - Category: Hematology Source Type: research
A 62-year-old man with acute myeloid leukemia, evolved from myelodysplastic syndrome after allogeneic bone marrow transplant, was admitted to the hospital because of worsening fatigue and altered level of consciousness. On arrival at the hospital, the patient was markedly fatigued and had signs of encephalopathy. He reported no fevers, chills, or diaphoresis but noted new urinary incontinence and nausea. His medical history was notable for a matched unrelated donor allogeneic hematopoietic stem cell transplant (HCT), performed 35 days previously, as well as hypertension and peripheral vascular disease.
Source: Mayo Clinic Proceedings - Category: Internal Medicine Authors: Tags: Residents' Clinic Source Type: research
We report a case of myeloid sarcoma with megakaryoblastic differentiation in the breast of a patient with history of essential thrombocythemia. The mass was composed of undifferentiated pleomorphic malignant cells in trabecular cords and nests with many scattered giant malignant cells and brisk abnormal mitoses. On immunohistochemistry, the neoplastic cells were positive for CD61, CD31, CD34, Factor VIII and CD43 which confirmed the diagnosis. To our knowledge, this is the first report of myeloid sarcoma with megakaryoblastic morphology occurring in the breast. Here we discuss the clinicopathologic features of this rare en...
Source: Hematology Oncology and Stem Cell Therapy - Category: Cancer & Oncology Source Type: research
Abstract Myelodysplastic syndrome (MDS) is a malignant hematopoietic stem cell disorder that frequently evolves into acute myeloid leukemia (AML). Patients with MDS are prone to infectious complications, in part due to the presence of severe neutropenia and/or neutrophil dysfunction. However, not all patients with neutropenia become infected, suggesting that other immune cells may compensate in these patients. Monocytes are also integral to immunologic defense; however, much less is known about monocyte function in patients with MDS. In the current study, we monitor the composition of peripheral blood monocytes an...
Source: Journal of Leukocyte Biology - Category: Hematology Authors: Tags: J Leukoc Biol Source Type: research
Contributors : Aniththa Thivakaran ; Lacra Botezatu ; Judith H önes ; Judith Schütte ; Lothar Vassen ; Nadine Olberding ; Yahya S Al-Matary ; Renata Köster ; Klaus Lennartz ; Andre Görgens ; Bernd Giebel ; Bertram Opalka ; Ulrich Dührsen ; Cyrus KhandanpourSeries Type : Genome binding/occupancy profiling by high throughput sequencingOrganism : Mus musculusDifferentiation of hematopoietic stem cells (HSCs) is regulated by a concert of different transcription factors (TFs). A disturbed function of TFs can be the basis of (pre)malignancies such as myelodysplastic syndrome (MDS) or acute myeloid leukem...
Source: GEO: Gene Expression Omnibus - Category: Genetics & Stem Cells Tags: Genome binding/occupancy profiling by high throughput sequencing Mus musculus Source Type: research
Contributors : Aniththa Thivakaran ; Lacra Botezatu ; Judith H önes ; Judith Schütte ; Lothar Vassen ; Nadine Olberding ; Yahya S Al-Matary ; Renata Köster ; Klaus Lennartz ; Andre Görgens ; Bernd Giebel ; Bertram Opalka ; Ulrich Dührsen ; Cyrus KhandanpourSeries Type : Expression profiling by arrayOrganism : Mus musculusDifferentiation of hematopoietic stem cells (HSCs) is regulated by a concert of different transcription factors (TFs). A disturbed function of TFs can be the basis of (pre)malignancies such as myelodysplastic syndrome (MDS) or acute myeloid leukemia (AML). Growth Factor Independenc...
Source: GEO: Gene Expression Omnibus - Category: Genetics & Stem Cells Tags: Expression profiling by array Mus musculus Source Type: research
Pediatric myelodysplastic syndromes (MDSs) are a heterogeneous group of clonal disorders with an annual incidence of 1 to 4 cases per million, accounting for less than 5% of childhood hematologic malignancies. MDSs in children often occur in the context of inherited bone marrow failure syndromes, which represent a peculiarity of myelodysplasia diagnosed in pediatric patients. Moreover, germ line syndromes predisposing individuals to develop MDS or acute myeloid leukemia have recently been identified, such as those caused by mutations in GATA2, ETV6, SRP72, and SAMD9/SAMD9-L. Refractory cytopenia of childhood (RCC) is the m...
Source: Blood - Category: Hematology Authors: Tags: Pediatric Hematology, Transplantation, How I Treat, Free Research Articles, Myeloid Neoplasia Source Type: research
Contributors : Hiroyoshi Kunimoto ; Cem Meydan ; Francine E Garrett-Bakelman ; Caroline Sheridan ; Tak Lee ; Yaseswini Neelamraju ; Ari Melnick ; Ross L LevineSeries Type : Methylation profiling by high throughput sequencingOrganism : Mus musculusRecent studies using next-generation sequencing technology have uncovered mutational landscapes of various myeloid malignancies (Cancer Genome Atlas Research Network, 2013; Yoshida et al., 2011). These genetic data revealed novel classes of mutations that commonly occur in patients with myeloid malignancies, including epigenetic regulators and spliceosomal genes. In addition, co-o...
Source: GEO: Gene Expression Omnibus - Category: Genetics & Stem Cells Tags: Methylation profiling by high throughput sequencing Mus musculus Source Type: research
Contributors : Hiroyoshi Kunimoto ; Cem Meydan ; Francine E Garrett-Bakelman ; Caroline Sheridan ; Tak Lee ; Yaseswini Neelamraju ; Ari Melnick ; Ross L LevineSeries Type : Expression profiling by high throughput sequencingOrganism : Mus musculusRecent studies using next-generation sequencing technology have uncovered mutational landscapes of various myeloid malignancies (Cancer Genome Atlas Research Network, 2013; Yoshida et al., 2011). These genetic data revealed novel classes of mutations that commonly occur in patients with myeloid malignancies, including epigenetic regulators and spliceosomal genes. In addition, co-oc...
Source: GEO: Gene Expression Omnibus - Category: Genetics & Stem Cells Tags: Expression profiling by high throughput sequencing Mus musculus Source Type: research
Abstract Patient-derived xenotransplantation models of human myeloid diseases including acute myeloid leukemia, myelodysplastic syndromes and myeloproliferative neoplasms are essential for studying the disease's biology in pre-clinical studies. However, few studies have used these models for comparison purposes. Previous work has shown that acute myeloid leukemia blasts respond to human hematopoietic cytokines whereas myelodysplastic syndrome cells do not. We compared the engraftment of acute myeloid leukemia cells and myelodyplastic syndrome cells in NSG mice to NSG-S mice, which have transgene expression of huma...
Source: Haematologica - Category: Hematology Authors: Tags: Haematologica Source Type: research
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