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International Journal of Hematology

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https://www.readbyqxmd.com/read/29149425/correction-to-efficacy-and-safety-of-full-length-pegylated-recombinant-factor-viii-with-extended-half-life-in-previously-treated-patients-with-hemophilia-a-comparison-of-data-between-the-general-and-japanese-study-populations
#1
Keiji Nogami, Midori Shima, Katsuyuki Fukutake, Teruhisa Fujii, Masashi Taki, Tadashi Matsushita, Satoshi Higasa, Tetsuji Sato, Michio Sakai, Morio Arai, Haruhiko Uchikawa, Werner Engl, Brigitt Abbuehl, Barbara A Konkle
The authors would like to correct the error in Table 2 in the original publication of the article. The "Blood type" is not described in any part of "Results" and "Discussion" and had no impact on the conclusion hence the bottom of the table is removed.
November 17, 2017: International Journal of Hematology
https://www.readbyqxmd.com/read/29147843/iron-and-infection
#2
REVIEW
Tomas Ganz
Iron is an essential trace metal for nearly all infectious microorganisms, and host defense mechanisms target this dependence to deprive microbes of iron. This review highlights mechanisms that are activated during infections to restrict iron on mucosal surfaces, in plasma and extracellular fluid, and within macrophages. Iron overload disorders, such as hereditary hemochromatosis or β-thalassemia, interfere with iron-restrictive host responses, and thereby cause increased susceptibility to infections with microbes that can exploit this vulnerability...
November 16, 2017: International Journal of Hematology
https://www.readbyqxmd.com/read/29143282/hypomethylating-agents-for-treatment-and-prevention-of-relapse-after-allogeneic-blood-stem-cell-transplantation
#3
REVIEW
Thomas Schroeder, Christina Rautenberg, Rainer Haas, Ulrich Germing, Guido Kobbe
Despite the curative potential of allogeneic stem cell transplantation (allo-SCT) in patients with acute myeloid leukemia (AML) and myelodysplastic syndromes (MDS), many patients will relapse. Until recently therapeutic options mainly consisted of palliative care, chemotherapy, donor lymphocyte infusions and second transplantation in selected cases. Still many patients either do not tolerate intensive therapies or do not achieve durable remissions and will finally succumb. Given this unmet medical need the hypomethylating agents (HMA), Azacitidine (Aza) and Decitabine (DAC) have been tested as salvage therapy in patients with myeloid malignancies relapsing after allo-SCT...
November 15, 2017: International Journal of Hematology
https://www.readbyqxmd.com/read/29143281/long-term-outcome-in-patients-treated-at-home-during-the-pancytopenic-phase-after-allogeneic-haematopoietic-stem-cell-transplantation
#4
Olle Ringdén, Behnam Sadeghi, Gianluca Moretti, Sigrun Finnbogadottir, Brita Eriksson, Jonas Mattsson, Britt-Marie Svahn, Mats Remberger
Patients undergoing allogeneic haematopoietic stem cell transplantation (HSCT) were given the option to be treated at home during the pancytopenic phase. Daily visits by a nurse and phone calls from a physician from the unit were part of the protocol. During almost two decades, 252 patients with haematological malignancies and non-malignant disorders were included. Median age was 47 (range 0-72) years. Myeloablative conditioning was given to 102 patients and reduced intensity to 150. Donors were matched unrelated (n = 160), HLA-identical siblings (n = 71), or HLA-mismatched (n = 21)...
November 15, 2017: International Journal of Hematology
https://www.readbyqxmd.com/read/29139060/iron-metabolism-in-erythroid-cells-and-patients-with-congenital-sideroblastic-anemia
#5
REVIEW
Kazumichi Furuyama, Kiriko Kaneko
Sideroblastic anemias are anemic disorders characterized by the presence of ring sideroblasts in a patient's bone marrow. These disorders are typically divided into two types, congenital or acquired sideroblastic anemia. Recently, several genes were reported as responsible for congenital sideroblastic anemia; however, the relationship between the function of the gene products and ring sideroblasts is largely unclear. In this review article, we will focus on the iron metabolism in erythroid cells as well as in patients with congenital sideroblastic anemia...
November 14, 2017: International Journal of Hematology
https://www.readbyqxmd.com/read/29134618/the-mechanisms-of-systemic-iron-homeostasis-and-etiology-diagnosis-and-treatment-of-hereditary-hemochromatosis
#6
REVIEW
Hiroshi Kawabata
Hereditary hemochromatosis (HH) is a group of genetic iron overload disorders that manifest with various symptoms, including hepatic dysfunction, diabetes, and cardiomyopathy. Classic HH type 1, which is common in Caucasians, is caused by bi-allelic mutations of HFE. Severe types of HH are caused by either bi-allelic mutations of HFE2 that encodes hemojuvelin (type 2A) or HAMP that encodes hepcidin (type 2B). HH type 3, which is of intermediate severity, is caused by bi-allelic mutations of TFR2 that encodes transferrin receptor 2...
November 13, 2017: International Journal of Hematology
https://www.readbyqxmd.com/read/29103138/a-new-prognostic-score-comprising-lactate-dehydrogenase-albumin-and-neutrophil-to-lymphocyte-ratio-to-predict-sensitivity-to-first-line-chemotherapy-in-patients-with-peripheral-t-cell-lymphomas
#7
Satoshi Kaito, Yusuke Kanemasa, Yuki Sasaki, Toshihiro Okuya, Tsukasa Yamaguchi, Chikako Funasaka, Tatsu Shimoyama, Yasushi Omuro, Tsunekazu Hishima, Yoshiharu Maeda
No standard therapy for peripheral T-cell lymphomas (PTCLs) has been established, and treatment outcomes are poor. Upfront stem cell transplantation has been investigated in several studies, some of which have reported promising outcomes. However, some patients maintain long-term remission after chemotherapy alone. It is thus important to predict sensitivity to first-line chemotherapy to optimize treatment strategies. In the present study, we retrospectively analyzed time to treatment failure (TTF) of first-line chemotherapy in 59 patients with PTCLs...
November 4, 2017: International Journal of Hematology
https://www.readbyqxmd.com/read/29098541/notch1-expression-is-regulated-at-the-post-transcriptional-level-by-the-3-untranslated-region-in-hematopoietic-stem-cell-development
#8
Shinichi Mizuno, Tadafumi Iino, Hidetoshi Ozawa, Yojiro Arinobu, Yong Chong, Koichi Akashi
In hematopoiesis, the expression of critical genes is regulated in a stage-specific manner to maintain normal hematopoiesis. Notch1 is an essential gene involved in the commitment and development of the T-cell lineage. However, the regulation of Notch1 in hematopoiesis is controversial, particularly at the level of hematopoietic stem cell (HSC). Here, we found that the expression of Notch1 is controlled at the post-transcriptional level in HSCs. HSCs express a considerable level of Notch1 mRNA, but its protein level is very low, suggesting a post-transcriptional suppression for Notch1...
November 2, 2017: International Journal of Hematology
https://www.readbyqxmd.com/read/29090417/long-term-clinical-remission-maintained-after-cessation-of-zidovudine-and-interferon-%C3%AE-therapy-in-chronic-adult-t-cell-leukemia-lymphoma
#9
Lucy B Cook, Aileen G Rowan, Maria A Demontis, Sophie Sagawe, Nicolas A Gillet, Anat Melamed, Claire Greiller, Aviva Witkover, Charles R M Bangham, Graham P Taylor
Globally, > 5-10 million people are estimated to be infected with Human T-lymphotropic virus type 1 (HTLV-1), of whom ~ 5% develop adult T-cell leukemia/lymphoma (ATL). Despite advances in chemotherapy, overall survival (OS) has not improved in the 35 years since HTLV-1 was first described. In Europe/USA, combination treatment with zidovudine and interferon-α (ZDV/IFN-α) has substantially changed the management of patients with the leukemic subtypes of ATL (acute or unfavorable chronic ATL) and is under clinical trial evaluation in Japan...
October 31, 2017: International Journal of Hematology
https://www.readbyqxmd.com/read/29076005/nilotinib-vs-imatinib-in-japanese-patients-with-newly-diagnosed-chronic-myeloid-leukemia-in-chronic-phase-long-term-follow-up-of-the-japanese-subgroup-of-the-randomized-enestnd-trial
#10
Hirohisa Nakamae, Tetsuya Fukuda, Chiaki Nakaseko, Yoshinobu Kanda, Ken Ohmine, Takaaki Ono, Itaru Matsumura, Akira Matsuda, Makoto Aoki, Kazuo Ito, Hirohiko Shibayama
In the ongoing, international, phase 3 study Evaluating Nilotinib Efficacy and Safety in Clinical Trials-Newly Diagnosed Patients (ENESTnd), nilotinib 300 and nilotinib 400 mg, both twice daily, are compared with imatinib 400 mg once daily for the treatment of newly diagnosed chronic myeloid leukemia in the chronic phase (CML-CP). Results for the overall population in ENESTnd (n = 846) showed that nilotinib resulted in higher response rates vs. imatinib and was well tolerated. Outcomes among Japanese patients in ENESTnd were specifically analyzed after 1 year of follow-up, and showed similar trends to the overall population; we present updated analysis of the Japanese subgroup based on 5 years of follow-up...
October 26, 2017: International Journal of Hematology
https://www.readbyqxmd.com/read/29071478/gene-analysis-of-inherited-antithrombin-deficiency-and-functional-analysis-of-abnormal-antithrombin-protein-n87d
#11
Sayaka Kamijima, Akiko Sekiya, Mao Takata, Haruka Nakano, Morika Murakami, Tomonori Nakazato, Hidesaku Asakura, Eriko Morishita
Inherited antithrombin (AT) deficiency is one of the most clinically significant forms of congenital thrombophilia and follows an autosomal dominant mode of inheritance. We analyzed SERPINC1 in a patient who developed deep-vein thrombosis and low AT activity during pregnancy, and identified a novel missense mutation c.259A>G (p.Asn87Asp; N87D). Surprisingly, analysis of the parents' DNA showed that they did not possess this mutant, and thus, it may have been due to a de novo mutation. We also expressed this mutant AT protein in COS-1 cells and compared its intracellular localization and intracellular and extracellular antigen levels with that of wild-type AT...
October 25, 2017: International Journal of Hematology
https://www.readbyqxmd.com/read/29071477/enforced-expression-of-mir142-a-target-of-chromosome-translocation-in-human-b-cell-tumors-results-in-b-cell-depletion
#12
Kodai Kuriyama, Yutaka Enomoto, Ritsuro Suzuki, Jyuri Watanuki, Hiroki Hosoi, Yusuke Yamashita, Shogo Murata, Toshiki Mushino, Shinobu Tamura, Nobuyoshi Hanaoka, Martin Dyer, Reiner Siebert, Hiroshi Kiyonari, Hideki Nakakuma, Toshio Kitamura, Takashi Sonoki
MicroRNA142 (MIR142) is a target of chromosome translocations and mutations in human B-cell lymphomas. We analyzed an aggressive B-cell lymphoma carrying t(8;17)(q24;q22) and t(6;14)(p21;q32), and sought to explore the role(s) of MIR142 in lymphomagenesis. t(8;17)(q24;q22) involved MYC on 8q24 and pri-MIR142 on 17q22. MYC was activated by a promoter substitution by t(8;17)(q24;q22). t(8;17)(q24;q22) was an additional event after t(6;14) (p21;q32), which caused the over-expression of CCND3. Southern blot analyses revealed that the MIR142 locus was deleted from the affected allele, whereas Northern analyses showed over-expression of MIR142 in tumor cells...
October 25, 2017: International Journal of Hematology
https://www.readbyqxmd.com/read/29067594/genetic-variation-of-kr%C3%A3-ppel-like-factor-1-klf1-and-fetal-hemoglobin-hbf-levels-in-%C3%AE-0-thalassemia-hbe-disease
#13
Pinyaphat Khamphikham, Orapan Sripichai, Thongperm Munkongdee, Suthat Fucharoen, Sissades Tongsima, Duncan R Smith
Heterogeneity of HbF levels in β(0)-thalassemia/HbE disease has been reported to be associated with variations in clinical manifestations of the disease, and several genetic-modifying factors beyond the β-globin gene cluster have been identified as HbF regulators. Down-regulation or heterozygous mutations of Krüppel-like factor 1 (KLF1) is associated with elevated HbF levels in non-thalassemia subjects. This study confirms that experimental down-regulation of KLF1 in β(0)-thalassemia/HbE-derived erythroblasts significantly increases HbF production (up to 52...
October 24, 2017: International Journal of Hematology
https://www.readbyqxmd.com/read/29067593/jsh-guideline-for-tumors-of-hematopoietic-and-lymphoid-tissues-leukemia-3-acute-lymphoblastic-leukemia-lymphoblastic-lymphoma-all-lbl
#14
Jin Takeuchi, Shigeru Kusumoto, Hideki Akiyama, Yoshinobu Kanda, Koji Izutsu
No abstract text is available yet for this article.
October 24, 2017: International Journal of Hematology
https://www.readbyqxmd.com/read/29052026/high-dose-chemotherapy-and-autologous-peripheral-blood-stem-cell-transplantation-with-bcvac-regimen-followed-by-maintenance-chemotherapy-for-children-with-very-high-risk-acute-lymphoblastic-leukemia
#15
Che Ry Hong, Hyoung Jin Kang, Kyung Duk Park, Hee Young Shin, Hyo Seop Ahn
Allogeneic hematopoietic stem cell transplantation (HSCT) is the recommended treatment for children with very high risk acute lymphoblastic leukemia (ALL), but it requires adequate institutional infrastructure, experience, and expertise, especially for alternative donor HSCT. We review our experience with high-dose chemotherapy (HDCT) and autologous peripheral blood stem cell transplantation (APBSCT), followed by post-APBSCT maintenance chemotherapy for children with very high risk ALL. Between August 1997 and November 2012, our institute was not successful with HLA-haploidentical HSCT...
October 20, 2017: International Journal of Hematology
https://www.readbyqxmd.com/read/29043551/magi-1-expression-is-decreased-in-several-types-of-human-t-cell-leukemia-cell-lines-including-adult-t-cell-leukemia
#16
Takashi Kozakai, Masahiko Takahashi, Masaya Higuchi, Toshifumi Hara, Kousuke Saito, Yuetsu Tanaka, Masayoshi Masuko, Jun Takizawa, Hirohito Sone, Masahiro Fujii
Membrane-associated guanylate kinase with inverted orientation protein 1 (MAGI-1) is a cytoplasmic scaffold protein that interacts with various signaling molecules; it negatively controls the cell growth of various types of cells and positively controls cell-cell interaction. In T cells, MAGI-1 has been shown to inhibit Akt activity through its interaction with PTEN and MEK1. In this study we found that MAGI-1 expression is decreased in multiple (9 out of 15) human T-cell leukemia cell lines, including adult T-cell leukemia (ATL), T-cell acute lymphoblastic leukemia and chronic T-cell lymphocytic leukemia...
October 17, 2017: International Journal of Hematology
https://www.readbyqxmd.com/read/29043550/collision-of-metastatic-malignant-melanoma-and-acute-myelogenous-leukemia-in-the-bone-marrow
#17
LETTER
Nobuhiko Kobayashi, Hiroaki Shimizu, Junko Hirato, Hiroshi Handa
No abstract text is available yet for this article.
October 17, 2017: International Journal of Hematology
https://www.readbyqxmd.com/read/29032513/sudden-blast-phase-in-chronic-myeloid-leukemia-developed-during-nilotinib-therapy-after-major-molecular-response-was-achieved
#18
Yosuke Okada, Ken Sato, Shinichi Kobayashi, Shigeki Nagao, Kosuke Takano, Masahiro Teramoto, Noriaki Tachi, Toshikuni Kawamura, Toshikatsu Horiuchi, Shoichiro Kato, Reina Saga, Takaaki Maekawa, Takeshi Yamamura, Junichi Watanabe, Ayako Kobayashi, Fumihiko Kimura
Sudden blast phase (SBP) is a rare event in which patients with chronic myeloid leukemia (CML) in complete cytogenetic response (CCyR) rapidly progress to the blast phase. Few patients on second-generation tyrosine kinase inhibitors (2nd TKIs) have been reported to develop SBP. Here, we report a 45-year-old man diagnosed with CML in the chronic phase in April 2008 and immediately started on imatinib therapy. He achieved CCyR 12 months after starting imatinib therapy. Imatinib was followed by treatment with the 2nd TKIs nilotinib and dasatinib from January 2011 to yield a better response...
October 14, 2017: International Journal of Hematology
https://www.readbyqxmd.com/read/29030834/prognostic-impact-of-minimal-disseminated-disease-and-immune-response-to-npm-alk-in-japanese-children-with-alk-positive-anaplastic-large-cell-lymphoma
#19
Yuka Iijima-Yamashita, Tetsuya Mori, Atsuko Nakazawa, Reiji Fukano, Tetsuya Takimoto, Masahito Tsurusawa, Ryoji Kobayashi, Keizo Horibe
The prognostic impact of minimal disseminated disease (MDD) and anti-anaplastic lymphoma kinase (ALK) antibody titer in children with ALK-positive anaplastic large cell lymphoma (ALCL) was reported by an Italian/German group. Here, we examine their prognostic value in Japanese children with ALK-positive ALCL. We evaluated nucleophosmin (NPM)-ALK transcripts in 60 patients at diagnosis by RT-PCR and real-time PCR (qPCR). The antibody titer was assessed in 35 patients. Fifty-two percent were MDD positive by RT-PCR and 37% had more than 10 copies of NPM-ALK per 10(4) copies of ABL (10NCNs) by qPCR...
October 13, 2017: International Journal of Hematology
https://www.readbyqxmd.com/read/29027647/dasatinib-associated-reversible-demyelinating-peripheral-polyneuropathy-in-a-case-of-chronic-myeloid-leukemia
#20
Takashi Ishida, Naoyuki Akagawa, Tomomi Miyata, Naomi Tominaga, Takahiro Iizuka, Masaaki Higashihara, Takahiro Suzuki, Koji Miyazaki
Tyrosine kinase inhibitors (TKIs) are essential for the treatment of chronic myeloid leukemia (CML). Adverse effects of dasatinib have been reported; however, few reports have highlighted the association between dasatinib and demyelinating peripheral neuropathy (DPN). We report a patient with CML who developed acute onset of DPN associated with dasatinib therapy. A 46-year-old Japanese woman was treated with dasatinib for 7 months after the diagnosis of CML and she achieved a major molecular response (MMR)...
October 13, 2017: International Journal of Hematology
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