Read by QxMD icon Read

Acta Haematologica

Supawadee Yamsri, Naruwat Pakdee, Goonnapa Fucharoen, Kanokwan Sanchaisuriya, Supan Fucharoen
Non-transfusion-dependent thalassemia (NTDT) is associated with various forms of thalassemia and genetic modifiers. We report the molecular basis of NTDT in hemoglobin (Hb) E-β-thalassemia disease. This study was done in 73 adult patients encountered at the prenatal diagnosis center of Khon Kaen University, Northeast Thailand. Hematological parameters and Hb patterns were collected, and α- and β-globin gene mutations were determined. Multiple single-nucleotide polymorphisms (SNPs) including the rs7482144/Gγ-XmnI polymorphism, rs2297339, rs2838513, rs4895441, and rs9399137 in the HBS1L-MYB gene, rs4671393 and rs11886868 in the BCL11A gene, and G176AfsX179 in the KLF1 gene were examined...
October 7, 2016: Acta Haematologica
Naoki Mori, Mari Ohwashi-Miyazaki, Michiko Okada, Kentaro Yoshinaga, Masayuki Shiseki, Junji Tanaka
No abstract text is available yet for this article.
October 7, 2016: Acta Haematologica
Murat Kose, Oguz Kagan Bakkaloglu, Shirkhan Amikishiyev, Timur Selcuk Akpınar, Basak Saracoglu, Tugce Akcan, Melike Oktem, Mustafa Nuri Yenerel, Kerim Güler, Tufan Tükek
Acquired hemophilia is a relatively rare clinical presentation, and most cases present with acquired FVIII inhibitor. The co-occurrence of inhibitors to multiple coagulation factors is uncommon. These autoantibodies may induce spontaneous life-threatening bleeding in patients who have had no previous bleeding disorder. Herein, we present a patient with postpartum acquired FVIII and FIX inhibitors who developed intramuscular hematoma and hemothorax during follow-up. She was then treated with activated prothrombin complex concentrate and methylprednisolone...
October 5, 2016: Acta Haematologica
Mohamed Shanshal, Andrew Shakespeare, Seshadri Thirumala, Boyd Fenton, Donald P Quick
Dasatinib is a potent inhibitor of the altered tyrosine kinase activity in disease states associated with BCR/ABL1. This agent has been shown to exhibit broad off-target kinase inhibition and immunomodulating properties. These effects may be responsible for dasatinib's unique side effects including a distinctive form of hemorrhagic colitis. We report a case of hemorrhagic colitis associated with dasatinib use in a patient with chronic myelogenous leukemia. Colon biopsies at the time of symptomatic colitis confirmed CD3+CD8+ T cell infiltration...
September 23, 2016: Acta Haematologica
Dan Gong, Wei Li, Liang-Ding Hu, Jian-Liang Shen, Mei-Yun Fang, Qing-Ming Yang, Heng-Xiang Wang, Xiao-Yan Ke, Hui-Ren Chen, Zhao Wang, Hui Liu, Feng Liu, Yi-Gai Ma, Jing-Wen Wang, Hong-Hua Li, Quan-Shun Wang, Yu Jing, Xiao-Ning Gao, Li-Ping Dou, Yong-Hui Li, Jian-Min Luo, Li Yu
BACKGROUND: The survival of patients with acute myeloid leukemia (AML) with t(8;21) was reported to be shorter in China than in other countries. PATIENTS: We analyzed the correlation between different cytarabine (Ara-c) regimens and outcome in 255 t(8;21) AML patients in China who received postremission consolidation chemotherapy only. RESULTS: The 5-year overall survival (OS) of the high-dose Ara-c group (HDAC; 2≤ Ara-c ≤3 g/m2), intermediate-dose Ara-c group (MDAC; 1...
September 17, 2016: Acta Haematologica
Muhamed Baljevic, Bogdan Dumitriu, Ju-Whei Lee, Elisabeth M Paietta, Peter H Wiernik, Janis Racevskis, Christina Chen, Eytan M Stein, Robert E Gallagher, Jacob M Rowe, Frederick R Appelbaum, Bayard L Powell, Richard A Larson, Steven E Coutré, Jeffrey Lancet, Mark R Litzow, Selina M Luger, Neal S Young, Martin S Tallman
Telomeres are the capping ends of chromosomes that protect the loss of genetic material and prevent chromosomal instability. In human tissue-specific stem/progenitor cells, telomere length (TL) is maintained by the telomerase complex, which consists of a reverse transcriptase catalytic subunit (TERT) and an RNA template (TERC). Very short telomeres and loss-of-function mutations in the TERT and TERC genes have been reported in acute myeloid leukemia, but the role of telomeres in acute promyelocytic leukemia (APL) has not been well established...
September 16, 2016: Acta Haematologica
Jérôme Cornillon, Marie Balsat, Aurélie Cabrespine, Emmanuelle Tavernier-Tardy, Eric Hermet, Aurélien Mulliez, Karine Augeul-Meunier, Denis Guyotat, Jacques-Olivier Bay
Reduced intensity conditioning for allogeneic hematopoietic stem cell transplantation (allo-HSCT) is often proposed for patients with comorbidities. To enhance engraftment and limit graft-versus-host disease (GVHD), antithymoglobulin (ATG) is usually used. However, the dose needed remains unclear unlike myeloablative conditioning. In order to clarify this point, we conducted a retrospective study on patients who received a reduced intensity conditioning allo-HSCT based on a 2-day fludarabine and busulfan treatment with either 1 or 2 days of ATG treatment...
September 14, 2016: Acta Haematologica
Burak Uz
No abstract text is available yet for this article.
2016: Acta Haematologica
Haa-Na Song, Seok Jin Kim, Young Hyeh Ko, Won Seog Kim
BACKGROUND: Mediastinal gray zone lymphoma (MGZL) shares clinical characteristics with primary mediastinal B-cell lymphoma (PMBCL) and nodular sclerosing Hodgkin lymphoma (NSHL). However, MGZL is extremely rare, and an appropriate treatment for it has not yet been established. METHODS: We retrospectively analyzed 8 patients who were treated with systemic chemotherapy for MGZL between 2007 and 2014. RESULTS: The patients with MGZL were predominantly young and male (median age 26 years), and 62...
2016: Acta Haematologica
Sandro Trova, Paolo Mereu, Elena Cocco, Bruno Masala, Laura Manca, Monica Pirastru
During a screening for hemoglobinopathies, we found a carrier of the Sardinian δβ-thalassemia condition. The proband's hematology and hemoglobin (Hb) profile agreed with those of the other carriers previously identified during our diagnostic program except for the fetal Hb (HbF) composition, which consisted of both α2Aγ2 and α2Gγ2 instead of nearly 100% α2Aγ2. In order to explain the unusual γ-chain ratio, sequencing of the Gγ promoter was carried out and revealed two nucleotide substitutions in cis: C→T at position -474 and A→G at position -309 from the Cap site...
2016: Acta Haematologica
Hiroaki Shimizu, Nobuhiko Kobayashi, Masahiro Mihara, Hirono Iriuchishima, Takuma Ishizaki, Yoshihisa Kojima, Hiroshi Handa
Epstein-Barr virus-associated lymphoproliferative disorder (EBV-LPD) is a currently emerging serious complication in immunosuppressed patients, especially in allogeneic transplant recipients. Several fatal cases of EBV-LPD have been reported in aplastic anemia (AA) patients receiving immunosuppressive therapy (IST) with antithymocyte globulin (ATG) plus cyclosporine A (CsA), but no appropriate prophylactic or therapeutic strategy has been established. Herein, we describe a 29-year-old man whose EBV-LPD was successfully treated with rituximab...
2016: Acta Haematologica
Erik Hulegårdh, Mari Punab, Erik Holmberg, Katrin Palk, Edward Laane, Hele Everaus, Lovisa Wennström, Dick Stockelberg
This study focuses on the incidence, treatment, and survival of de novo acute leukemia in a 25-year perspective in western Sweden and Estonia. At the beginning of our study, Estonia was a part of the Eastern bloc with planned economy, but since 1991 it is a member of the European Union and transforming into a market economy. Survival rates have steadily increased in both countries. However, a gap between their survival curves remains. Based on our data, it is difficult to explain the big difference in the 5-year relative survival in favor of western Sweden (55 vs...
2016: Acta Haematologica
Yoshimasa Kamoda, Kiyotaka Izumi, Futoshi Iioka, Takashi Akasaka, Fumihiko Nakamura, Chiyuki Kishimori, Katsuyo Tsuda, Katsuhiro Fukutsuka, Atsuko Okumura, Masahiko Hayashida, Hitoshi Ohno
Philadelphia chromosome-positive (Ph+) acute lymphoblastic leukemia (ALL) may include the lymphoid blast crisis of chronic myeloid leukemia (CML-BC). We applied fluorescence in situ hybridization (FISH) of the BCR-ABL fusion gene to peripheral blood and/or bone marrow smears to determine whether the fusion was restricted to mononuclear cell nuclei or if segmented cell nuclei representing mature neutrophils also carried the fusion (Seg-FISH). Among 20 patients with Ph+ ALL without a prior diagnosis of CML, 9 were Seg-FISH+ and 11 were Seg-FISH-...
2016: Acta Haematologica
Elena Balestri, Jorge Hugo Villafañe, Lucia Bertozzi, Stefano Berlini, Angiola Rocino, Anna Maria Paganoni, Lorenzo Drago, Pedro Berjano
BACKGROUND: The aim of this study was to provide an Italian version of the Haemophilia Activities List (HAL) and check its reliability in Italian medical centers. METHODS: The Italian version of this assessment was administered to 80 patients (aged 18-65 years) affected by haemophilia A and B (moderate or severe). The validation was accomplished by comparing it to the revised and expanded Arthritis Impact Measurement Scales (AIMS2). RESULTS: The internal consistency of the Italian version of the HAL had statistically high results: Cronbach's α 0...
2016: Acta Haematologica
Taiwo R Kotila
BACKGROUND: Sickle cell trait (SCT) is the heterozygous form of sickle cell disease and expectedly should be a benign state with no complications ascribed to it. There are numerous reports challenging its being a benign condition, though this is controversial. METHODS AND RESULTS: A review of the results of the accompanying investigations done on some of the patients show that beta thalassemia may be responsible for many of the ascribed symptoms and complications...
2016: Acta Haematologica
Aleksandra Leszczynska, Jolanta Grzenkowicz-Wydra, Lidia Chmielewska-Gorycka, Maria Bieniaszewska, Andrzej Hellmann
INTRODUCTION: The identification of mutations of the JAK2 gene is a useful marker in the diagnosis of polycythemia vera (PV) patients. We studied the frequency of JAK2 mutations in a group of PV patients because data are still very limited regarding this subject in Polish patients. METHODS: The JAK2 V617F mutation was examined using the amplification refractory mutation system (ARMS)-PCR method. Direct sequencing and a cloning technique were performed to determine alternations in exon 12 of the JAK2 gene...
2016: Acta Haematologica
Guo-Rong Wang, Wan-Jun Sun, Wen-Ming Chen, Zhong-Xia Huang, Jia-Jia Zhang, Na An, Man Shen, Xin Li
OBJECTIVES: The long-term clinical characteristics, response to therapy, and survival in patients with immunoglobulin D (IgD) multiple myeloma (MM) were investigated. METHODS: A retrospective study was conducted that included 68 patients treated in the last 10 years, 37 of whom received bortezomib only (bortezomib group), 13 of whom received bortezomib and underwent autologous hematopoietic stem cell transplantation (bortezomib + ASCT group), and 18 of whom received conditional chemotherapy (non-bortezomib group)...
2016: Acta Haematologica
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
A Girolami, E Cosi, C Santarossa, S Ferrari, A M Lombardi
OBJECTIVE: To evaluate the impact of an asymptomatic congenital clotting defect (FXII deficiency) versus that of a similar but symptomatic defect (FXI deficiency) on protection from thrombosis. PATIENTS AND METHODS: All patients with FXII or FXI deficiency and thrombosis were gathered from a time-unlimited PubMed search that was carried out twice and from personal records. Combined defects were excluded. The defect had to be proven by the demonstration of a suited hereditary pattern and by a specific clotting assay...
2016: Acta Haematologica
Ofir Wolach, Richard Stone
Autoimmune and inflammatory conditions (AICs) are encountered in up to 25% of patients with myelodysplastic syndromes (MDS). A wide range of AICs have been reported in association with MDS and can range from limited clinical manifestations to systemic diseases affecting multiple organs. Vasculitides, connective tissue diseases, and inflammatory arthritis are frequently reported in different studies; noninfectious fever and constitutional symptoms at presentation are common. Associations between AICs and specific MDS characteristics vary by study, but the available data suggest that AICs cluster more often in younger patients with higher-risk MDS...
2016: Acta Haematologica
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"