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A nationwide survey of hypoplastic myelodysplastic syndrome (a multicenter retrospective study)

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

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Publication date: Available online 12 August 2017 Source:Oral Surgery, Oral Medicine, Oral Pathology and Oral Radiology Author(s): Michelle Bongiorno, Shayna Rivard, Daniel Hammer, Joshua Kentosh Dyskeratosis congenita (DC) is a rare, inherited, bone marrow failure syndrome caused by premature telomere shortening. The classic mucocutaneous triad of clinical features is reticulated skin pigmentation, nail dysplasia, and oral leukoplakia. Multiple somatic features are also common including bone marrow failure, pulmonary fibrosis, and liver disease. DC significantly increases the risk for malignant transformation, including ...
Source: Oral Surgery, Oral Medicine, Oral Pathology and Oral Radiology - Category: ENT & OMF Source Type: research
Abstract Inherited bone marrow failure syndromes (IBMFS) are rare cancer predisposition syndromes with an especially high risk of transformation to myelodysplastic syndrome (MDS) and/or acute myeloid leukemia (AML). We performed a retrospective systematic review of reported MDS/AML arising in the eight most common IBMFS to determine the frequency and outcome of chromosome 7 abnormalities. We identified 738 MDS/AML cases of 4,293 individuals. Monosomy 7 or del (7q) occurred in ∼17%. Greater understanding of the roles played by sequential acquisition of genetic and cytogenetic changes will provide insights into myeloid l...
Source: Pediatric Blood and Cancer - Category: Cancer & Oncology Authors: Tags: REVIEW Source Type: research
opathic Bone Marrow Failure Syndromes Abstract The Japanese National Research Group on Idiopathic Bone Marrow Failure Syndromes has been conducting prospective registration, central review, and follow-up study for patients with aplastic anemia and myelodysplastic syndrome (MDS) since 2006. Using this database, we retrospectively analyzed the prognosis of patients with MDS. As of May 2016, 351 cases were registered in this database, 186 of which were eligible for the present study. Kaplan-Meier analysis showed that overall survival (OS) curves of the five risk categories stipulated by the revised international prog...
Source: International Journal of Hematology - Category: Hematology Authors: Tags: Int J Hematol Source Type: research
Inherited forms of myelodysplastic syndrome (MDS) and myeloid or lymphoid leukemia have long been associated with several constitutional disorders in children. The first report on an inherited bone marrow failure syndrome (IBMFS) with high risk for the development of myeloid neoplasia (MN) dates back to exactly 90 years ago when the Swiss pediatrician Guido Fanconi described a series of patients with physical anomalies and pernicious anemia. Since then a number of additional IBMFS and inherited conditions with leukemia predisposition such as Down syndrome, Ras-pathway disorders or Li-Fraumeni syndrome and other hereditary ...
Source: Seminars in Hematology - Category: Hematology Authors: Source Type: research
AbstractPurpose of ReviewAcute leukemia is the most common cancer of childhood. The pathogenesis of childhood leukemia is multifactorial, and most cases occur sporadically. Although the majority of genetic changes are acquired somatic mutations, recent discoveries have revealed that inherited germline mutations are present in an increasing proportion of children, predisposing them to leukemia.Recent FindingsRecently described germline mutations inETV6,SH2B3, andPAX5 have been associated with increased risks of acute lymphoblastic leukemia (ALL). A high incidence of germline mutations inGATA2 is seen in pediatric myelodyspl...
Source: Current Genetic Medicine Reports - Category: Genetics & Stem Cells Source Type: research
In this study, we tested a radiation-free conditioning regimen with a T-cell–depleted graft to eliminate radiation exposure and minimize early and late toxicities of transplant. Forty-five patients (median age, 8.2 years; range 4.3-44) with FA underwent HCT between June 2009 and May 2014. The preparative regimen included busulfan, cyclophosphamide, fludarabine, and rabbit anti-thymocyte globulin. Busulfan levels were monitored to avoid excess toxicity. All grafts were CD34-selected/T-cell–depleted using the CliniMacs CD34 columns (Miltenyi). Thirty-four patients (75.6%) with marrow failure and 11 (24.4%) with m...
Source: Blood - Category: Hematology Authors: Tags: Pediatric Hematology, Transplantation, Free Research Articles, Red Cells, Iron, and Erythropoiesis, Clinical Trials and Observations Source Type: research
The inherited marrow failure syndromes (IBMFS) are a heterogeneous group of diseases characterized by failure in the production of one or more blood lineage. The clinical manifestations of the IBMFS vary according to the type and number of blood cell lines involved, including different combinations of anemia, leukopenia, and thrombocytopenia. In some IBMFS, systemic non-hematologic manifestations, including congenital malformations, mucocutaneous abnormalities, developmental delay, and other medical complications, may be present.
Source: Seminars in Hematology - Category: Hematology Authors: Source Type: research
Abstract Essential facts Haematological malignancies are a diverse group of cancers that affect the blood, bone marrow and lymphatic systems. The main categories are lymphoma, leukaemia, myeloma, myelodysplastic syndromes and myeloproliferative neoplasms. In addition, there are subtypes of lymphoma and leukaemia, as well as more rare haematological cancers that have their own categories. There are also borderline conditions such as aplastic anaemia and other non-malignant bone marrow failure syndromes. The charity Bloodwise says 38,000 people in Britain are diagnosed every year with blood cancer or a related disor...
Source: Nursing Standard - Category: Nursing Authors: Tags: Nurs Stand Source Type: research
Causes of macrocytic anemia among 628 patients: mean corpuscular volumes of 114 and 130 fL as critical markers for categorization. Int J Hematol. 2016 Jun 28; Authors: Takahashi N, Kameoka J, Takahashi N, Tamai Y, Murai K, Honma R, Noji H, Yokoyama H, Tomiya Y, Kato Y, Ishizawa K, Ito S, Ishida Y, Sawada K, Harigae H Abstract There have been no studies on the distribution of causes of macrocytic anemia with respect to mean corpuscular volume (MCV) cutoff values. We retrospectively investigated the causes of macrocytic anemia (MCV ≥100 fL) among 628 patients who visited the outpatient hema...
Source: International Journal of Hematology - Category: Hematology Authors: Tags: Int J Hematol Source Type: research
Publication date: September–December 2014 Source:Best Practice & Research Clinical Haematology, Volume 27, Issues 3–4 Author(s): Blanche P. Alter Fanconi anemia (FA) is a rare autosomal recessive cancer-prone inherited bone marrow failure syndrome, due to mutations in 16 genes, whose protein products collaborate in a DNA repair pathway. The major complications are aplastic anemia, acute myeloid leukemia (AML), myelodysplastic syndrome (MDS), and specific solid tumors. A severe subset, due to mutations in FANCD1/BRCA2, has a cumulative incidence of cancer of 97% by age 7 years; the cancers are AML, brain ...
Source: Best Practice and Research Clinical Haematology - Category: Hematology Source Type: research
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