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International Journal of Hematology

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https://read.qxmd.com/read/30762219/efficacy-and-safety-of-tyrosine-kinase-inhibitors-for-newly-diagnosed-chronic-phase-chronic-myeloid-leukemia-over-a-5-year-period-results-from-the-japanese-registry-obtained-by-the-new-target-system
#1
Masahiro Kizaki, Naoto Takahashi, Noriyoshi Iriyama, Shinichiro Okamoto, Takaaki Ono, Noriko Usui, Koiti Inokuchi, Chiaki Nakaseko, Mineo Kurokawa, Masahiko Sumi, Fumihiko Nakamura, Tatsuya Kawaguchi, Ritsuro Suzuki, Kazuhito Yamamoto, Kazunori Ohnishi, Itaru Matsumura, Tomoki Naoe
We report the results of a multicenter observational study using the New TARGET system, in which the effectiveness and safety of tyrosine kinase inhibitors (TKIs) were evaluated in newly diagnosed chronic-phase chronic myeloid leukemia (CML) patients. A total of 506 patients were enrolled between April 2010 and March 2013. Median age was 56 (range 18-92) years; 35% of patients were females. As the first-line therapy, 139 (27.9%), 169 (33.9%) and 144 (28.9%) patients were treated with imatinib, nilotinib, and dasatinib, respectively...
February 14, 2019: International Journal of Hematology
https://read.qxmd.com/read/30758723/atypical-sifd-with-novel-trnt1-mutations-a-case-study-on-the-pathogenesis-of-b-cell-deficiency
#2
Eri Kumaki, Keisuke Tanaka, Kohsuke Imai, Yuki Aoki-Nogami, Akira Ishiguro, Satoshi Okada, Hirokazu Kanegane, Fumihiko Ishikawa, Tomohiro Morio
Mutation in the gene encoding tRNA nucleotidyl transferase, CCA-adding 1 (TRNT1), an enzyme essential for the synthesis of the 3'-terminal CCA sequence in tRNA molecules, results in a disorder that features sideroblastic anemia, B-cell immunodeficiency, periodic fever, and developmental delay. Mutations in TRNT1 are also linked to phenotypes including retinitis pigmentosa, cataracts, and cardiomyopathy. To date, it has remained unclear how defective TRNT1 is linked to B-cell deficiency. Here we report the case of a 12-year-old boy without sideroblastic anemia who harbors novel compound heterozygous mutations in TRNT1...
February 13, 2019: International Journal of Hematology
https://read.qxmd.com/read/30756344/potential-role-of-activated-factor-viii-fviiia-in-fviia-tissue-factor-dependent-fxa-generation-in-initiation-phase-of-blood-coagulation
#3
Shoko Furukawa, Keiji Nogami, Kenichi Ogiwara, Midori Shima
Factor VIIa/tissue factor (FVIIa/TF) initiates blood coagulation by promoting FXa generation (extrinsic-Xa). Subsequent generation of intrinsic FXa (intrinsic-Xa) amplifies thrombin formation. Previous studies suggested that FVIIa/TF activates FVIII rapidly in immediate coagulation reactions, and FVIIa/TF/FXa activates FVIII prior to thrombin-dependent feedback. We investigated FVIII/FVIIa/TF/FXa relationships in early coagulation mechanisms. Total FXa generated by FVIIa/TF and FVIIa/TF-activated FVIII (FVIIIaVIIa/TF ) was 22...
February 13, 2019: International Journal of Hematology
https://read.qxmd.com/read/30734904/feasibility-of-salvage-cord-blood-transplantation-using-a-fludarabine-melphalan-and-low-dose-anti-thymocyte-globulin-conditioning-regimen
#4
Takumi Hoshino, Satoru Takada, Nahoko Hatsumi, Toru Sakura
Primary graft failure (PGF) is a lethal complication that occurs early after allogeneic stem cell transplantation (allo-SCT). Cord blood transplantation (CBT) is a potential re-transplantation option. Total body irradiation (TBI) is often incorporated into the pre-salvage CBT conditioning regimen following PGF; however, patients experiencing PGF are not always amenable to TBI, and non-TBI regimens for salvage CBT should be established. Here, we report five patients with hematologic malignancies who received salvage CBT for PGF following a non-TBI regimen using fludarabine (Flu), melphalan (Mel), and low-dose anti-thymocyte globulin (ATG)...
February 8, 2019: International Journal of Hematology
https://read.qxmd.com/read/30725360/phase-ii-study-of-flagm-fludarabine-high-dose-cytarabine-granulocyte-colony-stimulating-factor-mitoxantrone-for-relapsed-or-refractory-acute-myeloid-leukemia
#5
Nahoko Hatsumi, Shuichi Miyawaki, Takahiro Yamauchi, Akihiro Takeshita, Norio Komatsu, Noriko Usui, Yukihiro Arai, Fumihiro Ishida, Takeshi Morii, Yasuhiko Kano, Michinori Ogura, Shinichiro Machida, Kazuhiro Nishii, Sumihisa Honda, Kazunori Ohnishi, Tomoki Naoe
Given the poor prognosis of patients with relapsed/refractory acute myeloid leukemia (AML), better therapy is needed. Fludarabine enhances the efficacy of Ara-C (cytarabine) by increasing intracellular Ara-C-triphosphate. The FLAG (fludarabine, high-dose Ara-C, supported with granulocyte colony-stimulating factor) regimen has been tested for use in AML patients by other investigators. In the phase II study reported here, we evaluated the efficacy and toxicity of FLAGM therapy (FLAG with mitoxantrone), further intensified by adding mitoxantrone, based on the results of a phase I study by our group...
February 6, 2019: International Journal of Hematology
https://read.qxmd.com/read/30725359/prognostic-values-of-increased-b7-family-proteins-in-haploidentical-hematopoietic-stem-cell-transplantation-patients-with-agvhd
#6
Biqi Zhou, Tanzhen Wang, Lei Lei, Yutong Lu, Li Zhang, Xiaowen Tang, Huiying Qiu, Aining Sun, Xueguang Zhang, Yang Xu, Depei Wu
It has been reported that B7H1 and B7H3 play a role in graft-versus-host disease (GVHD), the major cause of treatment-related mortality (TRM) in haploidentical hematopoietic stem cell transplantation (haplo-HSCT) patients; however, the prognostic value of these factors has not been defined. We retrospectively collected 64 haplo-HSCT patients in our hospital from 2013 to 2014, as well as 38 HLA-matched-HSCT patients during the same period as the control group. We analyzed B7H1, B7H3, PD1, soluble CD25, ST2 and TNFR1 at 0 day, + 7 days, + 14 days and + 28 days after HSCT...
February 6, 2019: International Journal of Hematology
https://read.qxmd.com/read/30706328/identification-of-a-novel-ccdc22-mutation-in-a-patient-with-severe-epstein-barr-virus-associated-hemophagocytic-lymphohistiocytosis-and-aggressive-natural-killer-cell-leukemia
#7
Yusuke Yamashita, Akinori Nishikawa, Yoshifumi Iwahashi, Masakazu Fujimoto, Izumi Sasaki, Hiroyuki Mishima, Akira Kinoshita, Hiroaki Hemmi, Nobuo Kanazawa, Kouichi Ohshima, Ken-Ichi Imadome, Shin-Ichi Murata, Koh-Ichiro Yoshiura, Tsuneyasu Kaisho, Takashi Sonoki, Shinobu Tamura
Aggressive natural killer cell leukemia (ANKL) is a rare neoplasm characterized by the systemic infiltration of Epstein-Barr virus (EBV)-associated NK cells, and rapidly progressive clinical course. We report the case of a 45-year-old man with intellectual disability who developed ANKL, and describe the identification of a novel genetic mutation of coiled-coil domain-containing 22 (CCDC22). He presented with persistent fever, severe pancytopenia, and hepatosplenomegary. Following bone marrow aspiration, numerous hemophagocytes were identified...
January 31, 2019: International Journal of Hematology
https://read.qxmd.com/read/30706327/deregulated-polycomb-functions-in-myeloproliferative-neoplasms
#8
REVIEW
Goro Sashida, Motohiko Oshima, Atsushi Iwama
Polycomb proteins function in the maintenance of gene silencing via post-translational modifications of histones and chromatin compaction. Genetic and biochemical studies have revealed that the repressive function of Polycomb repressive complexes (PRCs) in transcription is counteracted by the activating function of Trithorax-group complexes; this balance fine-tunes the expression of genes critical for development and tissue homeostasis. The function of PRCs is frequently dysregulated in various cancer cells due to altered expression or recurrent somatic mutations in PRC genes...
January 31, 2019: International Journal of Hematology
https://read.qxmd.com/read/30701467/thalidomide-maintenance-therapy-in-japanese-myeloma-patients-a-multicenter-phase-ii-clinical-trial-comet-study
#9
Hirokazu Murakami, Tetsuhiro Kasamatsu, Jun Murakami, Toru Kiguchi, Takeshi Kanematsu, Daisuke Ogawa, Hiroyuki Takamatsu, Hiroshi Handa, Shuji Ozaki, Hirokazu Miki, Takeshi Takahashi, Takaaki Takeo, Tatsuya Yamauchi, Takanobu Morishita, Hiroshi Kosugi, Kazuyuki Shimizu
A prospective, multicenter, phase II study was performed to assess the efficacy and safety of thalidomide maintenance therapy at different doses in Japanese multiple myeloma (MM) patients. This study included 34 patients (median age, 74 years) who were previously treated with not more than three prior therapies and whose response status was evaluated as at least stable disease. They were randomized into Group A (no maintenance; 12 patients), Group B (50 mg thalidomide maintenance; 12 patients), and Group C (100 mg thalidomide maintenance; 10 patients), respectively...
January 31, 2019: International Journal of Hematology
https://read.qxmd.com/read/30701466/hematopoietic-stem-cell-transplantation-in-children-and-adolescents-with-relapsed-or-refractory-b-cell-non-hodgkin-lymphoma
#10
Naoto Fujita, Ryoji Kobayashi, Yoshiko Atsuta, Fuminori Iwasaki, Junji Suzumiya, Yoji Sasahara, Masami Inoue, Katsuyoshi Koh, Tsukasa Hori, Hiroaki Goto, Tatsuo Ichinohe, Yoshiko Hashii, Koji Kato, Ritsuro Suzuki, Tetsuo Mitsui
We undertook a retrospective study using the national registry data of hematopoietic stem cell transplantation (HSCT) in Japan to investigate the effect of graft source, particularly autologous or allogeneic tissue, on the treatment outcome in patients aged less than 18 years with relapsed or refractory B-cell non-Hodgkin lymphoma (B-NHL). Survival analysis was conducted on 31 autologous HSCT (auto-HSCT) and 48 allogeneic HSCT (allo-HSCT) recipients between 1990 and 2013. The 5-year survival rates were significantly lower for allo-HSCT compared to auto-HSCT recipients (32% vs...
January 30, 2019: International Journal of Hematology
https://read.qxmd.com/read/30701465/jsh-practical-guidelines-for-hematological-malignancies-2018-ii-lymphoma-8-adult-t-cell-leukemia-lymphoma
#11
Kunihiro Tsukasaki, Takuya Fukushima
No abstract text is available yet for this article.
January 30, 2019: International Journal of Hematology
https://read.qxmd.com/read/30694451/prophylaxis-and-treatment-with-mycophenolate-mofetil-in-children-with-graft-versus-host-disease-undergoing-allogeneic-hematopoietic-stem-cell-transplantation-a-nationwide-survey-in-japan
#12
Nozomu Kawashima, Minako Iida, Ritsuro Suzuki, Takahiro Fukuda, Yoshiko Atsuta, Yoshiko Hashii, Masami Inoue, Masao Kobayashi, Hiromasa Yabe, Keiko Okada, Souichi Adachi, Yuki Yuza, Keisei Kawa, Koji Kato
We investigated the safety and efficacy of mycophenolate mofetil (MMF) in the prevention and treatment of graft-versus-host disease (GVHD) using a nationwide retrospective survey in Japanese children undergoing hematopoietic stem cell transplantation (HSCT). Overall, 141 children undergoing allogeneic HSCT for hematological malignancy (n = 84), non-malignancy (n = 52), and solid tumors (n = 5) were administered MMF orally (median 8 years; range 0-15 years; 89 males and 52 females) during 1995-2011...
January 29, 2019: International Journal of Hematology
https://read.qxmd.com/read/30689137/discontinuation-of-l-asparaginase-and-poor-response-to-prednisolone-are-associated-with-poor-outcome-of-etv6-runx1-positive-pediatric-b-cell-precursor-acute-lymphoblastic-leukemia
#13
Ikuya Usami, Toshihiko Imamura, Yoshihiro Takahashi, So-Ichi Suenobu, Daiichiro Hasegawa, Yoshiko Hashii, Takao Deguchi, Tsukasa Hori, Akira Shimada, Koji Kato, Eturou Ito, Akiko Moriya-Saito, Hirohide Kawasaki, Hiroki Hori, Keiko Yumura-Yagi, Junichi Hara, Atsushi Sato, Keizo Horibe
ETV6-RUNX1-positive B precursor acute lymphoblastic leukemia (B-ALL) is a common subtype of pediatric B-ALL that has shown excellent outcomes in contemporary clinical trials for pediatric B-ALL. Examinations of the possibility of reducing therapeutic intensity may thus be explored. This prospective study examined outcomes in 205 pediatric patients with ETV6-RUNX1-positive B-ALL uniformly treated following the Japan Association of Childhood Leukemia Study Group (JACLS) ALL-02 protocol. The JACLS ALL-02 protocol does not employ minimal residual disease detected by polymerase chain reaction (PCR-MRD)-based risk stratification; however, 4-year event-free survival (EFS) and overall survival (OS) were 94...
January 28, 2019: International Journal of Hematology
https://read.qxmd.com/read/30684252/comparison-of-neutropenia-profiles-in-different-treatment-protocols-for-acute-myeloid-leukemia-using-the-d-index
#14
Yasufumi Kawasaki, Shun-Ichi Kimura, Hirofumi Nakano, Kiyomi Mashima, Yuya Shirato, Shin-Ichiro Kawaguchi, Yumiko Toda, Shin-Ichi Ochi, Takashi Nagayama, Daisuke Minakata, Ryoko Yamasaki, Kaoru Morita, Masahiro Ashizawa, Chihiro Yamamoto, Kaoru Hatano, Kazuya Sato, Iekuni Oh, Shin-Ichiro Fujiwara, Ken Ohmine, Shinichi Kako, Kazuo Muroi, Yoshinobu Kanda
Neutropenia is a major risk factor for opportunistic infections in patients with acute myeloid leukemia (AML) who undergo chemotherapy. In the present study, we retrospectively compared the D-index, which reflects both the depth and duration of neutropenia, between two different chemotherapy regimens for AML. Sixty-seven patients with AML were included: 37 received an induction regimen of daunorubicin (DNR) and cytarabine followed by consolidation therapies consisting of standard-dose cytarabine (SDAC) and other antineoplastic agents; the remaining 30 received idarubicin (IDR) and cytarabine as remission induction therapy followed by high-dose cytarabine (HDAC)...
January 25, 2019: International Journal of Hematology
https://read.qxmd.com/read/30680670/a-case-of-double-refractory-multiple-myeloma-with-both-the-igh-mmset-fusion-protein-and-the-congenital-abnormality-t-11-22
#15
Rikio Suzuki, Takayuki Warita, Yoshihiko Nakamura, Yuka Kitamura, Yasuyuki Aoyama, Yoshiaki Ogawa, Hiroshi Kawada, Kiyoshi Ando
A 67-year-old female was referred to our hospital with a sternal fracture in March 2008. She received a diagnosis of multiple myeloma (MM) BJP-κ type (ISS stage III). G-banding karyotype revealed 46, XX, t(11;22)(q23.3;q11.2) (Hubacek, Gene 592:193-9, 2016), which was later confirmed to be congenital. After repeated rounds of chemotherapy with bortezomib and lenalidomide, she obtained a very good partial response in August 2014, and she was followed up with no treatment. However, she relapsed in February 2016...
January 24, 2019: International Journal of Hematology
https://read.qxmd.com/read/30680669/clinicopathological-features-of-tafro-syndrome-complicated-by-acquired-hemophilia-a-and-development-of-cardiopulmonary-arrest-that-were-successfully-treated-with-va-ecmo-and-tocilizumab
#16
Kei Suzuki, Takeshi Matsumoto, Yoshiaki Iwashita, Ken Ishikura, Masaki Fujioka, Hideo Wada, Naoyuki Katayama, Hiroshi Imai
TAFRO syndrome and acquired hemophilia A (AHA) are rare, life-threatening diseases; however, the relationship between these two diseases is unknown. A 25-year-old man was transferred to our hospital because of bleeding tendency accompanied by multiple organ failure with generalized edema, massive pleural effusion, and ascites. He was diagnosed with AHA. Bypass therapy for hemostasis and cyclophosphamide with prednisolone to eradicate possible inhibitors were provided. However, he suffered from cardiopulmonary arrest...
January 24, 2019: International Journal of Hematology
https://read.qxmd.com/read/30680668/renal-dysfunction-and-anemia-associated-with-long-term-imatinib-treatment-in-patients-with-chronic-myelogenous-leukemia
#17
Masatoshi Sakurai, Taku Kikuchi, Daiki Karigane, Hidenori Kasahara, Eri Matsuki, Risa Hashida, Yusuke Yamane, Ryohei Abe, Yuya Koda, Takaaki Toyama, Jun Kato, Takayuki Shimizu, Yuta Yokoyama, Sayo Suzuki, Tomonori Nakamura, Shinichiro Okamoto, Takehiko Mori
Knowledge of the toxicity profile of long-term treatment with imatinib is limited. In the present study, we sought to evaluate renal function and hemoglobin levels during long-term imatinib treatment. Eighty-two patients with chronic myelogenous leukemia in chronic phase who had been on imatinib for over 5 years were retrospectively analyzed. The mean estimated glomerular filtration rate (eGFR) was significantly decreased over 5 years (77 ± 17 to 62 ± 14 ml/min/1.73m², P < 0.001). Higher age and lower eGFR value at initiation of imatinib were significantly associated with development of renal dysfunction by multivariate analyses...
January 24, 2019: International Journal of Hematology
https://read.qxmd.com/read/30680667/clinical-features-at-transformation-in-adult-t-cell-leukemia-lymphoma-with-smoldering-and-chronic-types
#18
Hiroaki Taniguchi, Yoshitaka Imaizumi, Yumi Takasaki, Jun Nakashima, Takeharu Kato, Hidehiro Itonaga, Shinya Sato, Yasushi Sawayama, Koji Ando, Hiroo Hasegawa, Tomoko Hata, Yukiyoshi Moriuchi, Kunihiro Tsukasaki, Yasushi Miyazaki
Watchful waiting (WW) is among the treatment options indicated for patients with indolent adult T-cell leukemia-lymphoma (ATL). However, we previously showed that the long-term prognosis of patients with smoldering and chronic ATL is often worse than expected, with many undergoing transformation to aggressive ATL. To identify clinical features associated with transformation of smoldering/chronic ATL, we retrospectively analyzed the clinical features of 44 patients (14 smoldering and 30 chronic) who experienced transformation during WW...
January 24, 2019: International Journal of Hematology
https://read.qxmd.com/read/30680666/effect-of-antithymocyte-globulin-on-hla-mismatched-unrelated-transplantation
#19
REVIEW
Koji Kawamura
HLA 1-locus-mismatched unrelated donors (1MMUD) are often considered as alternative donors in allogeneic hematopoietic stem-cell transplantation (allo-HCT) when an HLA-matched related or unrelated donor is unavailable. However, HLA mismatch remains a major risk factor for acute and chronic graft-versus-host disease (GVHD). Antithymocyte globulin (ATG) has been used to prevent acute and chronic GVHD, and multiple studies have shown that use of ATG is associated with decreased acute and chronic GVHD, which is associated with improved QOL...
January 24, 2019: International Journal of Hematology
https://read.qxmd.com/read/30680665/optimal-dosage-of-methotrexate-for-gvhd-prophylaxis-in-umbilical-cord-blood-transplantation
#20
Yoshitaka Adachi, Kazutaka Ozeki, Shun Ukai, Ken Sagou, Nobuaki Fukushima, Akio Kohno
The combination of methotrexate (MTX) and a calcineurin inhibitor is widely used for GVHD prophylaxis in umbilical cord blood transplantation (UCBT). However, the optimal MTX dosage for GVHD prophylaxis in UCBT remains unclear. In the present study, we investigated the impact of MTX dosage on clinical outcomes following UCBT in a single-center retrospective study. Of 70 UCBT recipients included in this study, 37 received MTX at doses of 10 mg/m2 on day 1, and 7 mg/m2 on days 3 and 6 (low-dose MTX: LD-MTX), and 33 received MTX at doses of 15 mg/m2 on day 1, and 10 mg/m2 on days 3 and 6 (standard-dose MTX: SD-MTX), in addition to tacrolimus (TAC)...
January 24, 2019: International Journal of Hematology
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