Read by QxMD icon Read

Hypoplastic myelodysplastic syndrome

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
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...
August 4, 2016: Oncotarget
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
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
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
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
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
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
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
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
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
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
Robert P Hasserjian, Sa A Wang
Bone marrow diagnosis presents unique challenges: distinction between benign and neoplastic conditions may be subtle and the pathologist must effectively incorporate information from several morphologic modalities (trephine biopsy, aspirate smear, peripheral blood smear) as well as a myriad of ancillary testing results. In this Short Course, several difficult diagnostic scenarios will be explored using actual clinical cases that illustrate key points in the differential diagnosis of bone marrow diseases. These will include hypocellular bone marrow (in which distinction between hypoplastic myelodysplastic syndrome and aplastic anemia may be difficult), fibrotic bone marrow, and marrow lymphoid infiltrates, with discussion of both B-cell and T-cell lymphomas...
October 2014: Pathology
Ditte J A Løhmann, Henrik Hasle
BACKGROUND: Therapy-related myelodysplastic syndrome/acute myeloid leukemia (t-MDS/AML) is a feared long-term complication of pediatric cancer. Few osteosarcoma patients develop t-MDS/AML, but the frequency of hematological abnormalities after therapy is unknown. We reviewed biochemistry from osteosarcoma patients up to 3 years posttreatment. PROCEDURE: All children diagnosed with osteosarcoma at our department from 2006 to 2012 without relapse 1 month posttherapy were included (n=14)...
April 2015: Journal of Pediatric Hematology/oncology
B Serio, Am Risitano, V Giudice, N Montuori, C Selleri
Hypocellular or hypoplastic myelodysplastic syndromes (HMDS) are a distinct subgroup accounting for 10-15% of all MDS patients, that are characterized by the presence of bone marrow (BM) hypocellularity, various degree of dysmyelopoiesis and sometimes abnormal karyotype. Laboratory and clinical evidence suggest that HMDS share several immune-mediated pathogenic mechanisms with acquired idiopathic aplastic anemia (AA). Different immune-mediated mechanisms have been documented in the damage of marrow hematopoietic progenitors occurring in HMDS; they include oligoclonal expansion of cytotoxic T lymphocytes (CTLs), polyclonal expansion of various subtypes of T helper lymphocytes, overexpression of FAS-L and of the TNF-related apoptosis-inducing ligand (TRAIL), underexpression of Flice-like inhibitory protein long isoform (FLIPL) in marrow cells as well as higher release of Th1 cytokines, such as interferon-gamma (IFN-γ) and tumor necrosis factor-alpha (TNF-α)...
January 2014: Translational Medicine @ UniSa
Lijian Shao, Yingying Wang, Jianhui Chang, Yi Luo, Aimin Meng, Daohong Zhou
Due to improvements in early detection and treatment of cancer, the number of long-term cancer survivors is increasing. Unfortunately, these survivors are at increased risk for developing cancer treatment-related late effects, including ionizing radiation (IR)- and chemotherapy-induced long-term bone marrow (LT-BM) injury. Because LT-BM injury can deteriorate over time or after the patients receiving additional cancer treatment or undergoing autologous BM transplantation, it may eventually lead to the development of hypoplastic anemia or myelodysplastic syndrome...
October 2013: Translational Cancer Research
Dron Gauchan, Hamid Shaaban, David Gedeon, Michael Maroules
No abstract text is available yet for this article.
October 2014: Annals of Hematology
Jun Shi, Yi-zhou Zheng
No abstract text is available yet for this article.
October 2013: Zhonghua Xue Ye Xue za Zhi, Zhonghua Xueyexue Zazhi
Gisela Caceres, Kathy McGraw, Bon Ham Yip, Andrea Pellagatti, Joseph Johnson, Ling Zhang, Kenian Liu, Lan Min Zhang, William J Fulp, Ji-Hyun Lee, Najla H Al Ali, Ashley Basiorka, Larry J Smith, F Joseph Daugherty, Neil Littleton, Richard A Wells, Lubomir Sokol, Sheng Wei, Rami S Komrokji, Jacqueline Boultwood, Alan F List
Stabilization of p53 in erythroid precursors in response to nucleosomal stress underlies the hypoplastic anemia in myelodysplastic syndromes (MDS) with chromosome 5q deletion [del(5q)]. We investigated whether cenersen, a clinically active 20-mer antisense oligonucleotide complementary to TP53 exon10, could suppress p53 expression and restore erythropoiesis in del(5q) MDS. Cenersen treatment of ribosomal protein S-14-deficient erythroblasts significantly reduced cellular p53 and p53-up-regulated modulator of apoptosis expression compared with controls, accompanied by a significant reduction in apoptosis and increased cell proliferation...
October 1, 2013: Proceedings of the National Academy of Sciences of the United States of America
Ryota Nakanishi, Mitsuaki Ishida, Keiko Hodohara, Takashi Yoshida, Miyuki Yoshii, Hiroko Okuno, Akiko Horinouchi, Muneo Iwai, Keiko Yoshida, Akiko Kagotani, Hidetoshi Okabe
Gelatinous bone marrow transformation (GMT) is a rare disorder characterized by the presence of fat cell atrophy, loss of hematopoietic cells, and deposition of extracellular gelatinous materials. GMT is not a specific disease, but is strongly associated with malnutrition and drugs. Albeit extremely rare, GMT has been reported in patients with myeloproliferative disorders. Herein, we report the second documented case of hypoplastic myelodysplastic syndrome (MDS) accompanying GMT. A 73-year-old Japanese male with excellent nutrition status and no history of alcohol or drug intake was detected with pancytopenia...
2013: International Journal of Clinical and Experimental Pathology
Fetch more papers »
Fetching more papers... Fetching...
Read by QxMD. Sign in or create an account to discover new knowledge that matter to you.
Remove bar
Read by QxMD icon Read

Search Tips

Use Boolean operators: AND/OR

diabetic AND foot
diabetes OR diabetic

Exclude a word using the 'minus' sign

Virchow -triad

Use Parentheses

water AND (cup OR glass)

Add an asterisk (*) at end of a word to include word stems

Neuro* will search for Neurology, Neuroscientist, Neurological, and so on

Use quotes to search for an exact phrase

"primary prevention of cancer"
(heart or cardiac or cardio*) AND arrest -"American Heart Association"