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Hypoplastic myelodysplastic syndrome

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https://www.readbyqxmd.com/read/28554891/dna-repair-gene-expressions-are-related-to-bone-marrow-cellularity-in-myelodysplastic-syndrome
#1
Howard L Ribeiro, Allan Rodrigo S Maia, Roberta Taiane G de Oliveira, Marília Braga Costa, Izabelle Rocha Farias, Daniela de Paula Borges, Juliana Cordeiro de Sousa, Silvia Maria M Magalhães, Ronald F Pinheiro
OBJECTIVE: To evaluate the expression of genes related to nuclear excision (ERCC8, XPA and XPC), homologous recombination and non-homologous end-joining (ATM, BRCA1, BRCA2 and LIG4) repair mechanisms, using quantitative PCR methodologies, and it relation with bone marrow cellularity in myelodysplastic syndrome (MDS). METHODS AND RESULTS: A total of 51 adult de novo patients with MDS (3 refractory anaemia (RA), 11 refractory anaemia with ringed sideroblasts (RARS), 28 refractory cytopenia with multilineage dysplasia (RCMD), 3 refractory anaemia with excess blasts type I (RAEB-I), 5 refractory anaemia with excess blasts type II (RAEB-II), and 1 chronic myelomonocytic leukaemia (CMML) were evaluated...
May 29, 2017: Journal of Clinical Pathology
https://www.readbyqxmd.com/read/28484160/rapidly-progressive-hemorrhagic-pneumonia-caused-by-stenotrophomonas-maltophilia-occurring-early-after-bone-marrow-transplantation-in-a-patient-with-myelodysplastic-syndrome
#2
Oju Katayama, Tomoya Kitagawa, Takashi Onaka, Akihito Yonezawa
A 44-year-old woman had repeated bacterial infections because of hypoplastic myelodysplastic syndrome; therefore, she underwent allogeneic hematopoietic cell transplantation. Broad spectrum antibiotics were administered because she had bacterial infection and pneumonia 2 weeks before undergoing transplantation. On day19 after transplantation, she suddenly presented with hemoptysis. Stenotrophomonas maltophilia was considered to be the cause of hemorrhagic pneumonia, which required ventilation support, massive transfusion, and catecholamine administration...
2017: [Rinshō Ketsueki] the Japanese Journal of Clinical Hematology
https://www.readbyqxmd.com/read/28472728/new-polymorphisms-of-xeroderma-pigmentosum-dna-repair-genes-in-myelodysplastic-syndrome
#3
Sabrina Pinheiro Santiago, Howard Lopes Ribeiro Junior, Juliana Cordeiro de Sousa, Daniela de Paula Borges, Roberta Taiane Germano de Oliveira, Izabelle Rocha Farias, Marília Braga Costa, Allan Rodrigo Soares Maia, Mayumi da Nóbrega Ito, Silvia Maria Meira Magalhães, Ronald Feitosa Pinheiro
The association between Xeroderma Pigmentosum DNA repair genes (XPA rs1800975, XPC rs2228000, XPD rs1799793 and XPF rs1800067) polymorphisms and myelodysplastic syndrome (MDS) have not been reported. To assess the functional role between these polymorphisms and MDS, we evaluated 189 samples stratified in two groups: 95 bone marrow samples from MDS patients and 94 from healthy elderly volunteers used as controls. Genotypes for all polymorphisms were identified in DNA samples in an allelic discrimination experiment by real-time polymerase chain reaction (qPCR)...
July 2017: Leukemia Research
https://www.readbyqxmd.com/read/28406545/diagnosing-nocturnal-paroxysmal-hemoglobinuria-a-single-center-4-year-experience
#4
T Mercier, T Devos, M Mukovnikova, N Boeckx
INTRODUCTION: Paroxysmal nocturnal hemoglobinuria (PNH) is a rare disease and can present as a wide range of signs and symptoms. As such, the indication for diagnostic testing for PNH is not always straightforward. Therefore, we analyzed all first-time samples tested over a 56-month period to determine the clinical settings with a high probability of detecting a PNH clone. METHODS: We retrospectively analyzed 323 first-time PNH flow cytometry tests, including LDH, cytopenias, direct antiglobulin test (DAT), and clinical indication for testing as available at the time of testing...
June 2017: International Journal of Laboratory Hematology
https://www.readbyqxmd.com/read/28059092/bone-marrow-morphology-and-disease-progression-in-congenital-thrombocytopenia-a-detailed-clinicopathologic-and-genetic-study-of-eight-cases
#5
Hamilton C Tsang, James B Bussel, Susan Mathew, Yen-Chun Liu, Allison A Imahiyerobo, Attilio Orazi, Julia T Geyer
Patients with congenital thrombocytopenia have an increased risk of developing myeloid neoplasms. In these cases, the morphologic distinction between disease at baseline and at progression is challenging. This report analyzes clinicopathologic features of congenital thrombocytopenia with long-term follow-up at one referral center. Records from the last 20 years were searched for cases of congenital thrombocytopenia with bone marrow biopsies and peripheral blood smears. The clinical, morphologic, immunophenotypic, and molecular features were analyzed...
April 2017: Modern Pathology: An Official Journal of the United States and Canadian Academy of Pathology, Inc
https://www.readbyqxmd.com/read/27913534/myelodysplastic-and-myeloproliferative-disorders-of-childhood
#6
REVIEW
Henrik Hasle
Myelodysplastic syndrome (MDS) and myeloproliferative disorders are rare in children; they are divided into low-grade MDS (refractory cytopenia of childhood [RCC]), advanced MDS (refractory anemia with excess blasts in transformation), and juvenile myelomonocytic leukemia (JMML), each with different characteristics and management strategies. Underlying genetic predisposition is recognized in an increasing number of patients. Germ line GATA2 mutation is found in 70% of adolescents with MDS and monosomy 7. It is challenging to distinguish RCC from aplastic anemia, inherited bone marrow failure, and reactive conditions...
December 2, 2016: Hematology—the Education Program of the American Society of Hematology
https://www.readbyqxmd.com/read/27913517/treatment-of-low-risk-myelodysplastic-syndromes
#7
REVIEW
Valeria Santini
The majority of myelodysplastic syndrome (MDS) patients belong to the International Prognostic Scoring System (IPSS) and IPSS-revised (IPSS-R) lower-risk categories. Their precise diagnostics and prognostic stratification is often a challenge, but may ensure the optimization of therapy. The availability of diverse treatment options has significantly improved the quality of life and survival of this group of patients. Anemia is the most relevant cytopenia in terms of frequency and symptoms in lower-risk MDS, and may be treated successfully with erythropoietic stimulating agents, provided a careful selection is performed on the basis of IPSS-R, endogenous erythropoietin levels, and transfusion independence...
December 2, 2016: Hematology—the Education Program of the American Society of Hematology
https://www.readbyqxmd.com/read/27784383/-analysis-of-lymphocyte-subsets-in-peripheral-blood-of-patients-with-aplastic-anemia-or-hypoplastic-myelodysplastic-syndrome
#8
Yuan-Fei Lv, Zheng-Yu Yan, Nai-Yiao Chen, Jian-Hui Wu, Bin Liu, Guo-Li Wang, Jing Wang
OBJECTIVE: To explore the ratio of lymphocyte subsets in peripheral blood of patients with aplastic anemia (AA) and patients with hypoplastic myelodysplastic syndrome (hypo-MDS) patients and to evaluate their significance. METHODS: The clinical data of 181 cases of AA and 111 cases of hypo-MDS from January 2008 to December 2014 were collected from Blood Diseases Hospital of Chinese academy of medical sciences, and then the differences of lymphocyte subsets and its effect in 2 groups were analyzed...
October 2016: Zhongguo Shi Yan Xue Ye Xue za Zhi
https://www.readbyqxmd.com/read/27743175/evaluation-of-efficacy-of-alemtuzumab-in-5-patients-with-aplastic-anemia-and-or-myelodysplastic-neoplasm
#9
Wolfgang Füreder, Sabine Cerny-Reiterer, Wolfgang R Sperr, Leonhard Müllauer, Eva Jäger, Ilse Schwarzinger, Klaus Geissler, Peter Valent
Patients with aplastic anemia or hypoplastic myelodysplastic syndrome (MDS) may respond to immunosuppressive therapy, including the anti-CD52 antibody alemtuzumab. We analyzed treatment responses to alemtuzumab in 5 patients with MDS or aplastic anemia (AA) evolving to MDS. Two patients with hypoplastic MDS (hMDS) showed a partial response (PR) to alemtuzumab. In both responding patients, a concomitant paroxysmal nocturnal hemoglobinuria (PNH) clone was detected before therapy. One responder relapsed after 15 months and underwent successful allogeneic stem cell transplantation...
October 14, 2016: Wiener Klinische Wochenschrift
https://www.readbyqxmd.com/read/27527853/distinct-mutation-profile-and-prognostic-relevance-in-patients-with-hypoplastic-myelodysplastic-syndromes-h-mds
#10
Chi-Yuan Yao, Hsin-An Hou, Tzung-Yi Lin, Chien-Chin Lin, Wen-Chien Chou, Mei-Hsuan Tseng, Ying-Chieh Chiang, Ming-Chih Liu, Chia-Wen Liu, Yuan-Yeh Kuo, Shang-Ju Wu, Xiu-Wen Liao, Chien-Ting Lin, Bor-Shen Ko, Chien-Yuan Chen, Szu-Chun Hsu, Chi-Cheng Li, Shang-Yi Huang, Ming Yao, Jih-Luh Tang, Woei Tsay, Chieh-Yu Liu, Hwei-Fang Tien
Myelodysplastic syndromes (MDS) are a heterogeneous group of hematologic malignancies. Although most MDS patients have normal or increased BM cellularity (NH-MDS), some have hypocellular BM (h-MDS). The reports concerning the differences in genetic alterations between h-MDS and NH-MDS patients are limited. In this study, 369 MDS patients diagnosed according to the WHO 2008 criteria were recruited. h-MDS patients had lower PB white blood cell and blast counts, and lower BM blast percentages, than those with NH-MDS...
September 27, 2016: Oncotarget
https://www.readbyqxmd.com/read/27521325/myelodysplastic-syndromes-in-adolescent-young-adults-one-institution-s-experience
#11
Joanna Grabska, Bijal Shah, Damon Reed, Najla Al Ali, Eric Padron, Hanadi Ramadan, Jeffrey Lancet, Alan List, Rami Komrokji
INTRODUCTION: There has been little improvement in cancer survival of adolescent and young adult (AYA) patients, aged 18 to 39 years, possibly reflecting different disease biology. Myelodysplastic syndrome (MDS) is mainly a disease of the elderly. The characteristics, outcomes, and response to treatment are not well described in the AYA population. PATIENTS AND METHODS: This was a retrospective review of patients from the Moffitt Cancer Center MDS database. We compared baseline characteristics and outcomes of the AYA population to older patients...
August 2016: Clinical Lymphoma, Myeloma & Leukemia
https://www.readbyqxmd.com/read/27409595/comparable-allogeneic-hematopoietic-cell-transplantation-outcome-of-a-haplo-identical-family-donor-with-an-alternative-donor-in-adult-aplastic-anemia
#12
COMMENT
Hawk Kim, Je-Hwan Lee, Young-Don Joo, Sung-Hwa Bae, Sang Min Lee, Jae-Cheol Jo, Yunsuk Choi, Jung-Hee Lee, Dae-Young Kim, Hun Mo Ryoo, Kyoo-Hyung Lee
We performed a study on allogeneic hematopoietic cell transplantation (alloHCT) from an HLA-haplo-identical familial donor (haploFD) using a busulfan-fludarabine-antithymocyte globulin conditioning regimen for severe aplastic anemia (sAA) and hypoplastic myelodysplastic syndrome. For the comparison between a haploFD and an alternative donor (AD; matched unrelated or partially matched donor) for sAA in adults, we collected haploFD data retrospectively and prospectively. Forty-eight AD cases were selected for the comparison with 16 haploFD cases...
2016: Acta Haematologica
https://www.readbyqxmd.com/read/27158585/isavuconazole-as-salvage-therapy-for-mucormycosis
#13
Bianca Graves, C Orla Morrissey, Andrew Wei, John Coutsouvelis, Samantha Ellis, Alan Pham, Julian Gooi, Michelle Ananda-Rajah
Mucormycosis carries a high mortality rate with few therapeutic options available. We describe a man with pulmonary/splenic mucormycosis complicating hypoplastic myelodysplastic syndrome on a background of chronic kidney disease, who achieved a complete response with salvage isavuconazole therapy following intolerance of consecutive courses of liposomal amphotericin and posaconazole therapy.
March 2016: Medical Mycology Case Reports
https://www.readbyqxmd.com/read/26939774/the-current-perspective-of-low-grade-myelodysplastic-syndrome-in-children
#14
REVIEW
Daisuke Hasegawa
Myelodysplastic syndrome (MDS) without increased blasts, i.e., low-grade MDS, is the most common subtype of pediatric MDS and has characteristics different from adult form of the disease. Although histological findings of bone marrow (BM) biopsies suggest that low-grade MDS is a morphologically distinctive entity, a subset of pediatric low-grade MDS may clinically overlap with aplastic anemia (AA), such as high likelihood of hypocellular marrow and normal karyotype. In addition, children with low-grade MDS are as likely to respond to immunosuppressive therapy as those with AA, which indicates that a part of these disorders might share a common pathogenesis, that is, T cell-mediated inhibition of hematopoiesis...
April 2016: International Journal of Hematology
https://www.readbyqxmd.com/read/26273061/comparison-of-long-term-outcomes-between-children-with-aplastic-anemia-and-refractory-cytopenia-of-childhood-who-received-immunosuppressive-therapy-with-antithymocyte-globulin-and-cyclosporine
#15
MULTICENTER STUDY
Asahito Hama, Yoshiyuki Takahashi, Hideki Muramatsu, Masafumi Ito, Atsushi Narita, Yoshiyuki Kosaka, Masahiro Tsuchida, Ryoji Kobayashi, Etsuro Ito, Hiromasa Yabe, Shouichi Ohga, Akira Ohara, Seiji Kojima
The 2008 World Health Organization classification proposed a new entity in childhood myelodysplastic syndrome, refractory cytopenia of childhood. However, it is unclear whether this morphological classification reflects clinical outcomes. We retrospectively reviewed bone marrow morphology in 186 children (median age 8 years; range 1-16 years) who were enrolled in the prospective study and received horse antithymocyte globulin and cyclosporine between July 1999 and November 2008. The median follow-up period was 87 months (range 1-146 months)...
November 2015: Haematologica
https://www.readbyqxmd.com/read/26273060/genomic-patterns-associated-with-hypoplastic-compared-to-hyperplastic-myelodysplastic-syndromes
#16
LETTER
Aziz Nazha, David Seastone, Tom Radivoyevitch, Bartlomiej Przychodzen, Hetty E Carraway, Bhumika J Patel, Jennifer Carew, Hideki Makishima, Mikkael A Sekeres, Jaroslaw P Maciejewski
No abstract text is available yet for this article.
November 2015: Haematologica
https://www.readbyqxmd.com/read/26228212/quantitative-detection-of-id4-gene-aberrant-methylation-in-the-differentiation-of-myelodysplastic-syndrome-from-aplastic-anemia
#17
Mian-Yang Li, Yuan-Yuan Xu, Hui-Yuan Kang, Xin-Rong Wang, Li Gao, Jian Cen, Wei Wang, Nan Wang, Yong-Hui Li, Li-Li Wang, Li Yu
BACKGROUND: The diagnosis of myelodysplastic syndrome (MDS), especially hypoplastic MDS, and MDS with low blast counts or normal karyotype may be problematic. This study characterized ID4 gene methylation in patients with MDS and aplastic anemia (AA). METHODS: The methylation status of ID4 was analyzed by bisulfite sequencing polymerase chain reaction (PCR) and quantitative real-time methylation-specific PCR (MethyLight PCR) in 100 patients with MDS and 31 patients with AA...
August 5, 2015: Chinese Medical Journal
https://www.readbyqxmd.com/read/26052501/successful-pregnancy-outcome-in-paroxysmal-nocturnal-hemoglobinuria-pnh-following-escalated-eculizumab-dosing-to-control-breakthrough-hemolysis
#18
Ruby Sharma, Alla Keyzner, Johnson Liu, Thomas Bradley, Steven L Allen
Pregnancy in women with paroxysmal nocturnal hemoglobinuria (PNH) is associated with increased maternal and fetal morbidity and mortality. There is limited published experience regarding therapy of PNH during pregnancy. We describe a case of a 30 year old female with hypoplastic myelodysplastic syndrome and PNH. After two years of treatment with eculizumab, she became pregnant. She developed breakthrough hemolysis at 20 weeks gestation. Pharmacokinetic and pharmacodynamic studies demonstrated a subtherapeutic eculizumab level with absence of complement blockade...
2015: Leukemia Research Reports
https://www.readbyqxmd.com/read/25466263/distinct-effect-of-cyclophosphamide-and-cyclosporine-on-pure-red-cell-aplasia-associated-with-t-cell-large-granular-lymphocyte-leukemia
#19
Rie Tabata, Chiharu Tabata
Large granular lymphocyte (LGL) leukemia is characterized by a clonal proliferation of large-sized lymphocytes with prominent large azurophilic cytoplasmic granules. Although most cases of T-LGL leukemia are indolent and asymptomatic during the course of the disease, some present with pure red cell aplasia (PRCA) and require therapy. We here reported a case of T-LGL leukemia complicated by PRCA in which anemia was resistant to cyclosporine and had been controlled for several years by cyclophosphamide; however, progressive anemia developed despite the administration of cyclophosphamide, but was ameliorated by the re-administration of cyclosporine...
December 2014: International Immunopharmacology
https://www.readbyqxmd.com/read/25295231/pp2a-the-achilles-heal-in-mds-with-5q-deletion
#20
REVIEW
David A Sallman, Sheng Wei, Alan List
Myelodysplastic syndromes (MDS) represent a hematologically diverse group of myeloid neoplasms, however, one subtype characterized by an isolated deletion of chromosome 5q [del(5q)] is pathologically and clinically distinct. Patients with del(5q) MDS share biological features that account for the profound hypoplastic anemia and unique sensitivity to treatment with lenalidomide. Ineffective erythropoiesis in del(5q) MDS arises from allelic deletion of the ribosomal processing S-14 (RPS14) gene, which leads to MDM2 sequestration with consequent p53 activation and erythroid cell death...
2014: Frontiers in Oncology
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