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  1. Diagnosis and management of acquired aplastic anemia in childhood. Guidelines from the Marrow Failure Study Group of the Pediatric Haemato-Oncology Italian Association (AIEOP)

    ... Journal Title:  Blood Cells Mol Dis Primary Author:  ... in the past 2decades because of advances in hematopoietic stem cell transplantation, immunosuppressive and biologic drugs, and ...

    Research Article last updated 07/20/2018 - 5:15pm.

  2. A systematic modeling study on the pathogenic role of p38 MAPK activation in myelodysplastic syndromes

    ... syndromes (MDS) are a group of clonal hematopoietic stem cell diseases. In addition to intrinsic genetic alterations, the effects ... protein kinase (MAPK) signaling pathway in hematopoietic cells are associated with the ineffective hematopoiesis in MDS. ...

    Research Article last updated 07/20/2018 - 5:14pm.

  3. Oral Azacitidine (CC-486) for the Treatment of Myelodysplastic Syndromes and Acute Myeloid Leukemia

    ... therapy for patients with AML not eligible for stem cell transplant , and as maintenance therapy for patients with MDS or ... of azacitidine by increasing exposure to cycling malignant cells. In early phase trials, oral azacitidine (CC-486) in extended dosing ...

    Research Article last updated 07/20/2018 - 5:15pm.

  4. Guidelines for the diagnosis and monitoring of paroxysmal nocturnal hemoglobinuria and related disorders by flow cytometry.

    ... hemoglobinuria (PNH) is a rare hematopoietic stem cell disorder characterized by a somatic mutation in the PIGA ... flow cytometry is the method of choice for identifying cells deficient in GPI-linked proteins and is, therefore, necessary for the ...

    Research Article last updated 07/20/2018 - 5:14pm.

  5. Outcome of Aplastic Anemia in adolescence. A survey of the Severe Aplastic Anemia Working Party of the European Group for Blood and Marrow Transplantation

    ... comparing (i) matched family donor hematopoietic stem cell transplantation performed as first line treatment with (ii) ... transplant given for failure and with (iii) Hematopoietic Stem Cell Transplantation performed after failed front-line immunosuppressive ... stem cell transplantation using bone marrow cells is the first choice treatment. If such a donor is not available, ...

    Research Article last updated 07/20/2018 - 5:15pm.

  6. Clinical roundtable monograph: Paroxysmal nocturnal hemoglobinuria: a case-based discussion.

    ... glycan anchor (PIG-A) in a multipotent hematopoietic stem cell (HSC), with clonal expansion of the mutated HSC. The mutation causes ... in the synthesis of glycosylphosphatidylinositol (GPI). In cells derived from normal HSCs, the complement regulatory proteins CD55 and ...

    Research Article last updated 07/20/2018 - 5:15pm.

  7. Paroxysmal nocturnal hemoglobinuria from bench to bedside

    ... therapy. PNH originates from a multipotent hematopoietic stem cell (HSC) that acquires a somatic mutation in a gene called ... regulatory proteins; their absence on the surface of PNH cells leads to complement-mediated hemolysis . The release of free ...

    Research Article last updated 07/20/2018 - 5:14pm.

  8. Myelodysplastic syndromes: revisiting the role of the bone marrow microenvironment in disease pathogenesis

    ... likely depends on interplay between aberrant hematopoietic cells and their microenvironment. How niche cells play a role in disease ... is poorly defined, but the delineation of the hematopoietic stem cell niche and the ability to interrogate its role in hematopoietic ...

    Research Article last updated 07/20/2018 - 5:14pm.

  9. The role of epigenetics in the regulation of apoptosis in myelodysplastic syndromes and acute myeloid leukemia.

    ... Disordered stem cell epigenetics and apoptosis -regulating mechanisms contribute ... its association with autophagy are upregulated in neoplastic cells in patients with low-risk MDS and epigenetically silenced and ...

    Research Article last updated 07/20/2018 - 5:15pm.

  10. Deregulation of innate immune and inflammatory signaling in myelodysplastic syndromes

    ... or constitutively activated in bone marrow hematopoietic stem and progenitor cells. Mounting experimental results indicate that the dysregulation of these ...

    Research Article last updated 07/20/2018 - 5:15pm.