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Myelodisplasia

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https://www.readbyqxmd.com/read/29521225/role-of-haematopoietic-stem-cell-transplantation-in-management-of-severe-chronic-neutropenia
#1
Francesca Fioredda
The inherited form of Severe Chronic Neutropenia called Severe Congenital Neutropenia (SCN) is an intrinsic defect of neutrophils maturation. SCN affected patients are at risk to develop lethal in-fections and to transform into myelodisplasia (MDS) and/or acute laeukemia(AL). Use of Granulocyte-Colony-Stimulating Factor (G-CSF) has significantly improved the prognosis of affected patients, but the only definitive cure for the disease is Haematopoietic Stem Cell Trans-plantation (HSCT). Overall Survival of SCN patients has been assessed at around 85% after 3 years from transplantation ...
March 8, 2018: Current Drug Targets
https://www.readbyqxmd.com/read/29217778/constitutional-samd9l-mutations-cause-familial-myelodysplastic-syndrome-and-transient-monosomy-7
#2
Victor B Pastor, Sushree S Sahoo, Jessica Boklan, Georg C Schwabe, Ebru Saribeyoglu, Brigitte Strahm, Dirk Lebrecht, Matthias Voss, Yenan T Bryceson, Miriam Erlacher, Gerhard Ehninger, Marena Niewisch, Brigitte Schlegelberger, Irith Baumann, John C Achermann, Akiko Shimamura, Jochen Hochrein, Ulf Tedgård, Lars Nilsson, Henrik Hasle, Melanie Boerries, Hauke Busch, Charlotte M Niemeyer, Marcin W Wlodarski
Familial myelodysplastic syndromes arise from haploinsufficiency of genes involved in hematopoiesis and are primarily associated with early-onset disease. Here we describe a familial syndrome in seven patients from four unrelated pedigrees presenting with myelodysplastic syndrome and loss of chromosome 7/7q. Their median age at diagnosis was 2.1 years (range, 1-42). All patients presented with thrombocytopenia with or without additional cytopenias and a hypocellular marrow without an increase of blasts. Genomic studies identified constitutional mutations (p...
March 2018: Haematologica
https://www.readbyqxmd.com/read/24075222/world-health-organization-classification-of-tumors-of-hematopoietic-and-lymphoid-tissues-4th-edition-2008-major-changes-from-the-3rd-edition-2001
#3
Maria Claudia Nogueira Zerbini, Fernando Augusto Soares, Elvira Deolinda Rodrigues Pereira Velloso, Maria de Lourdes L F Chaufaille, Roberto Pinto Paes
The World Health Organization (WHO) Classification of tumors of hematopoietic and lymphoid tissues (4th edition, 2008)1 presents an updated version of the 3rd edition published in 2001(2). A summary of these changes relates to the groups of chronic myeloproliferative disorders, myelodisplasia, acute myeloid leukemias, neoplasms of precursor B and T cells and neoplasms derived of mature B, T and NK cells. A better understanding of molecular genetic changes and results achieved with innovative therapeutic approaches in these groups of diseases requires constant reassessment of the classifications, supporting the major changes discussed here, including interesting comments from literature (1, 3-5)...
2011: Revista da Associação Médica Brasileira
https://www.readbyqxmd.com/read/8962805/-the-first-year-of-life-of-children-with-myelodysplasia-a-multicenter-study-of-393-cases
#4
MULTICENTER STUDY
M García Mérida, C Miguélez Lago, A Marqués Gubern, J García Romero, A López Martínez, R Isnart
A multicentric study about the first year of life of myelodisplastic children, was done. Family, pregnancy, delivery, newborn somatometry, and anatomical-pathological and terapeuthical aspects were review in 393 patients born before july 1992. Annual prevalence vary from 6 (1973) to 28 (1984). The families clinical history was not significant. There were 9 (2,3%) gemelar pregnancies, but both children were affected only in one case. Prenatal ultrasound was not diagnostic in 61%-84% of patients are meningomyeloceles, the anatomical level of myelodisplasia was lumbar and or sacral in 98%...
January 1996: Cirugía Pediátrica: Organo Oficial de la Sociedad Española de Cirugía Pediátrica
https://www.readbyqxmd.com/read/8815636/-levels-of-complement-components-in-plasma-of-onco-hematologic-patients-with-defective-hemopoiesis
#5
L P Rybakova, I V Tiagotin, A A Ganapiev, S I Moiseev, E A Ushakova, K M Abdulkadyrov, A M Ishchenko
Immunochemical assay using monoclonal antibodies was carried out to determine levels of a number of complement components (C3 and its derivatives-C4 and C5) and immunoglobulins (lg) in plasma of patients with onco-hematological diseases involving defective hemopoiesis: leukemia, myelodysplastic syndrome (clonal diseases) or aplastic anemia (delayed clonal disease). The most significant disorders were registered in the concentrations of component C3 and its derivatives. In acute leukemia, the nature and extent of C3 splitting was found to depend on disease while Ig level-on stage...
1996: Voprosy Onkologii
https://www.readbyqxmd.com/read/8525207/-allogenic-bone-marrow-transplantation-in-the-treatment-of-malignant-hematologic-disease
#6
F Barriga, P Bertín, R Baeza, J Pereira, L Schwartzmann, J Oliva, M Ocqueteau, F Sapunar, M Duhalde, P Lira
We have treated 28 patients (pts) with malignant hematological diseases with allogeneic bone marrow transplantation (BMT). 18 pts had acute lymphoblastic (ALL) and non lymphoblastic leukemia (ANLL), 5 chronic myeloid leukemia (CML), 2 severe aplastic anemia (SAA), 1 myelodisplasia, 1 Fanconi's anemia and 1 advanced Non Hodgkin's lymphoma. All but three received the graft from HLA identical sibling donors. We used conditioning with total body irradiation and chemotherapy (cyclophosphamide, cytarabine and etoposide) in 17 pts and chemotherapy alone in 11...
May 1995: Revista Médica de Chile
https://www.readbyqxmd.com/read/7667942/-morphologic-features-of-bone-marrow-in-myelodysplastic-states
#7
V I Rugal', V M Ponomarenko, T A Shatseva, K M Abdulkadyrov
The results of histological and ultrastructural studies of bone marrow hemopoietic microenvironment carried out in 27 patients with different myelodisplasias are presented. The following morphological peculiarities of bone marrow stromal tissue have been identified: abnormal localisation of immature hemopoietic cells, reticuline fibrosis, disorganized erythroid islands, and intranuclear virus-like pathologic inclusions in all stromal cells. The structure of nucleoli in endosteal and perivascular cells was changed...
1995: Voprosy Onkologii
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