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International Journal of Hematology

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https://www.readbyqxmd.com/read/27921272/bcl6-locus-is-hypermethylated-in-angioimmunoblastic-t-cell-lymphoma
#1
Shoko Nishizawa, Mamiko Sakata-Yanagimoto, Keiichiro Hattori, Hideharu Muto, Tran Nguyen, Koji Izutsu, Kenichi Yoshida, Seishi Ogawa, Naoya Nakamura, Shigeru Chiba
BCL6, a master transcription factor for differentiation of follicular helper T (TFH) cells, is highly expressed in angioimmunoblastic T-cell lymphoma (AITL) and peripheral T-cell lymphomas (PTCL) containing tumor cells with TFH features. TET2, encoding an epigenetic regulator, is frequently mutated in AITL/PTCL. We previously reported that Tet2 knockdown mice developed T-cell lymphomas with TFH features. Hypermethylation of the Bcl6 locus followed by BCL6 upregulation was thought to be the key event for lymphoma development in mice...
December 5, 2016: International Journal of Hematology
https://www.readbyqxmd.com/read/27921271/leaving-ijh-joining-to-fly
#2
EDITORIAL
Toshio Kitamura, Akifumi Takaori-Kondo
No abstract text is available yet for this article.
December 5, 2016: International Journal of Hematology
https://www.readbyqxmd.com/read/27914067/5q-syndrome-like-features-as-the-first-manifestation-of-myelodysplastic-syndrome-in-a-patient-with-an-unbalanced-whole-arm-translocation-der-5-19-p10-q10
#3
Hiroshi Ureshino, Haruna Kizuka, Kana Kusaba, Haruhiko Sano, Atsujiro Nishioka, Takero Shindo, Yasushi Kubota, Toshihiko Ando, Kensuke Kojima, Shinya Kimura
Derivative (5;19)(p10;q10) [der(5;19)(p10;q10)] is a rare chromosomal abnormality in myelodysplastic syndrome (MDS), and is genetically similar to deletion 5q [del(5q)]. However, MDS with der(5;19)(p10;q10) and 5q- syndrome are generally characterized as distinct subtypes. Here, we report a case of a patient with 5q- syndrome-like features as the first manifestation of MDS with der(5; 19)(p10;q10). A 59-year-old woman was admitted to our hospital for anemia without leukopenia and thrombocytopenia. She had received chemotherapy comprising carboplatin and docetaxel for endometrial cancer eight years before...
December 2, 2016: International Journal of Hematology
https://www.readbyqxmd.com/read/27910004/dabigatran-overdose-a-case-report-of-acute-hepatitis-extracorporeal-treatment
#4
Mariagrazia Porru, Antonella Mameli, Maria E Cianchetti, Mario Musu, Paola Schirru, Maria F Ruberto, Doris Barcellona, Francesco Marongiu
Dabigatran is an oral, direct thrombin inhibitor approved by international regulatory agencies for stroke prevention in patients with paroxysmal or persistent non-rheumatic atrial fibrillation (AF). The benefits of dabigatran are widely described, but its use in the geriatric population is not without risk. Chronic kidney disease is a common comorbidity with AF, and thus frequent checks of renal function in elderly patients are recommended. We report a case of dabigatran intoxication in an elderly man affected by heart failure and worsening renal function, who developed acute hepatitis and coma, which was successfully treated with continuous veno-venous hemodiafiltration...
December 1, 2016: International Journal of Hematology
https://www.readbyqxmd.com/read/27910003/bone-marrow-t-cell-percentage-a-novel-prognostic-indicator-in-acute-myeloid-leukemia
#5
Manar M Ismail, Nahla A B Abdulateef
Acute myeloid leukemia (AML) is an aggressive malignancy for which overall disease-free survival is less than 50%. Manipulation of the immune system is an interesting and promising therapy for AML patients. We aimed to characterize the immune system of AML patients, highlighting the clinical relevance of total bone marrow (BM) lymphocytes and subpopulations. Sixty-six new AML cases diagnosed according to WHO criteria from King Abdullah Medical City, KSA, from October 2012 to February 2015. Analysis of BM lymphocytes and subpopulations was done by flowcytometry...
December 1, 2016: International Journal of Hematology
https://www.readbyqxmd.com/read/27905003/new-agents-in-hsc-mobilization
#6
REVIEW
Mélanie J Domingues, Susan K Nilsson, Benjamin Cao
Mobilized peripheral blood (PB) is the most common source of hematopoietic stem cells (HSC) for autologous transplantation. Granulocyte colony stimulating factor (G-CSF) is the most commonly used mobilization agent, yet despite its widespread use, a considerable number of patients still fail to mobilize. Recently, a greater understanding of the interactions that regulate HSC homeostasis in the bone marrow (BM) microenvironment has enabled the development of new molecules that mobilize HSC through specific inhibition, modulation or perturbation of these interactions...
November 30, 2016: International Journal of Hematology
https://www.readbyqxmd.com/read/27900638/bendamustine-plus-rituximab-for-previously-untreated-patients-with-indolent-b-cell-non-hodgkin-lymphoma-or-mantle-cell-lymphoma-a-multicenter-phase-ii-clinical-trial-in-japan
#7
Michinori Ogura, Kenichi Ishizawa, Dai Maruyama, Naokuni Uike, Kiyoshi Ando, Koji Izutsu, Yasuhito Terui, Yoshitaka Imaizumi, Kunihiro Tsukasaki, Kenshi Suzuki, Tohru Izumi, Kensuke Usuki, Tomohiro Kinoshita, Masafumi Taniwaki, Nobuhiko Uoshima, Junji Suzumiya, Mitsutoshi Kurosawa, Hirokazu Nagai, Toshiki Uchida, Noriko Fukuhara, Ilseung Choi, Ken Ohmachi, Go Yamamoto, Kensei Tobinai
A Phase II, multicenter clinical trial of bendamustine plus rituximab (BR) regimen was conducted in previously untreated patients with high-tumor-burden indolent B-cell non-Hodgkin lymphoma (B-NHL) and previously untreated elderly patients with mantle cell lymphoma (MCL) in Japan. Bendamustine 90 mg/m(2)/day on days 1 and 2, as well as rituximab 375 mg/m(2) on day 1 were administered intravenously up to six cycles. The primary endpoint was the complete response (CR) rate as assessed by the International Workshop Response Criteria (1999)...
November 29, 2016: International Journal of Hematology
https://www.readbyqxmd.com/read/27896572/identification-of-novel-mutations-in-hfe-hfe2-tfr2-and-slc40a1-genes-in-chinese-patients-affected-by-hereditary-hemochromatosis
#8
Yongwei Wang, Yali Du, Gang Liu, Shanshan Guo, Bo Hou, Xianyong Jiang, Bing Han, Yanzhong Chang, Guangjun Nie
Hereditary hemochromatosis (HH) is a group of inherited iron-overload disorders associated with pathogenic defects in the genes encoding hemochromatosis (HFE), hemojuvelin (HJV/HFE2), hepcidin (HAMP), transferrin receptor 2 (TfR2), and ferroportin (FPN1/SLC40A1) proteins, and the clinical features are well described. However, there have been only a few detailed reports of HH in Chinese populations. Thus, there is insufficient patient information for population-based analyses in Chinese populations or comparative studies among different ethical groups...
November 28, 2016: International Journal of Hematology
https://www.readbyqxmd.com/read/27896571/erratum-to-japanese-phase-ii-study-of-rituximab-maintenance-for-untreated-indolent-b-cell-non-hodgkin-lymphoma-with-high-tumor-burden
#9
Tadahiko Igarashi, Michinori Ogura, Kuniaki Itoh, Masafumi Taniwaki, Kiyoshi Ando, Yoshiaki Kuroda, Kazuhito Yamamoto, Naokuni Uike, Akihiro Tomita, Hirokazu Nagai, Mitsutoshi Kurosawa, Shigeo Mori, Shigeru Nawano, Takashi Terauchi, Yasuo Ohashi, Kensei Tobinai
No abstract text is available yet for this article.
November 28, 2016: International Journal of Hematology
https://www.readbyqxmd.com/read/27882485/pharmacodynamics-of-t-cell-function-for-monitoring-pharmacologic-immunosuppression-after-allogeneic-hematopoietic-stem-cell-transplantation
#10
Carmen Martínez, Olga Millán, Montserrat Rovira, Francesc Fernández-Avilés, Anna López, María Suárez-Lledó, Enric Carreras, Álvaro Urbano-Ispízua, Mercè Brunet
Information on pharmacodynamic monitoring after allogeneic hematopoietic cell transplantation (allo-SCT) to evaluate individual responses to immunosuppressive drugs is scarce. We studied the relationship between a panel of pharmacodynamic markers monitored during the first 3 months after transplant and the occurrence of graft-versus-host disease (GVHD). Lymphocyte activation assessed by intracellular ATP concentration in CD4(+) T cells, a high percentage of CD8(+) effector T cells, and a low percentage of CD4(+) regulatory T (Treg) cells correlated significantly with GVHD...
November 23, 2016: International Journal of Hematology
https://www.readbyqxmd.com/read/27882484/diagnostic-challenge-of-diamond-blackfan-anemia-in-mothers-and-children-by-whole-exome-sequencing
#11
Takuya Ichimura, Kenichi Yoshida, Yusuke Okuno, Toshiaki Yujiri, Kozo Nagai, Masanori Nishi, Yuichi Shiraishi, Hiroo Ueno, Tsutomu Toki, Kenichi Chiba, Hiroko Tanaka, Hideki Muramatsu, Toshiro Hara, Hitoshi Kanno, Seiji Kojima, Satoru Miyano, Etsuro Ito, Seishi Ogawa, Shouichi Ohga
Diamond-Blackfan anemia (DBA) is a pure red cell aplasia that arises from defective ribosomal proteins (RPs). Patients with this rare ribosomopathy present with neonatal anemia and occasional dysmorphism. Clinical heterogeneity and clusters of causative RP genes hamper the diagnosis and perinatal management. We report three mother-and-child pairs of anemia who were finally diagnosed by whole-exome sequencing. Each pair showed distinct disease severity and response to anemia treatment. Only one mother had the diagnostic dysmorphism, including short stature, webbed neck, and thenar hypoplasia...
November 23, 2016: International Journal of Hematology
https://www.readbyqxmd.com/read/27873177/eculizumab-treatment-improved-renal-hemosiderosis-in-a-patient-with-paroxysmal-nocturnal-hemoglobinuria
#12
LETTER
Seiko Iki, Kazuyuki Ohgi, Kenshi Suzuki
No abstract text is available yet for this article.
November 21, 2016: International Journal of Hematology
https://www.readbyqxmd.com/read/27873176/palonosetron-aprepitant-and-dexamethasone-for-prevention-of-nausea-and-vomiting-after-high-dose-melphalan-in-autologous-transplantation-for-multiple-myeloma-a-phase-ii-study
#13
Atsushi Isoda, Rie Saito, Fuminori Komatsu, Yuki Negishi, Noriyasu Oosawa, Tetsuya Ishikawa, Yuri Miyazawa, Morio Matsumoto, Morio Sawamura, Akihiro Manaka
Chemotherapy-induced nausea and vomiting (CINV) is a significant side effect in multiple myeloma (MM) patients receiving high-dose melphalan treatment followed by autologous stem cell transplantation (ASCT). We evaluated the efficacy and safety of a triple antiemetic combination of palonosetron, aprepitant, and low-dose dexamethasone in 24 MM patients who received melphalan conditioning (100 mg/m(2) on days 1-2) before ASCT (on day 4). Intravenous palonosetron (0.75 mg on day 1), oral aprepitant (125 mg on day 1; 80 mg on days 2-4), and intravenous dexamethasone (6...
November 21, 2016: International Journal of Hematology
https://www.readbyqxmd.com/read/27873175/mobilization-of-human-immature-hematopoietic-progenitors-through-combinatory-use-of-bortezomib-and-immunomodulatory-drugs
#14
Taro Tochigi, Takatoshi Aoki, Yoshikane Kikushige, Tomohiko Kamimura, Yoshikiyo Ito, Takahiro Shima, Takuji Yamauchi, Yasuo Mori, Goichi Yoshimoto, Kenjiro Kamezaki, Koji Kato, Katsuto Takenaka, Hiromi Iwasaki, Koichi Akashi, Toshihiro Miyamoto
Combination use of the proteasome inhibitor bortezomib and the immunomodulatory drugs lenalidomide or thalidomide has provided superior outcomes in multiple myeloma over their single use; however, these combinations can produce significant toxicities. Unexpectedly, we found a small but significant increase in the population of immature granulocytes and erythrocytes/megakaryocytes in peripheral blood in 16 of 22 patients (73%) treated with dexamethasone in combination with bortezomib and immunomodulatory drugs (triplet), but not in any of 25 patients treated with either bortezomib or immunomodulatory drugs with dexamethasone (doublet)...
November 21, 2016: International Journal of Hematology
https://www.readbyqxmd.com/read/27858332/causative-genetic-mutations-for-antithrombin-deficiency-and-their-clinical-background-among-japanese-patients
#15
Akiko Sekiya, Fumina Taniguchi, Daisuke Yamaguchi, Sayaka Kamijima, Shonosuke Kaneko, Shiori Katsu, Miho Hanamura, Mao Takata, Haruka Nakano, Hidesaku Asakura, Shigeki Ohtake, Eriko Morishita
We summarize causative genetic mutations for antithrombin (AT) deficiency and their clinical background in Japanese patients. A total of 19 mutations, including seven novel mutations, were identified. We also summarize clinical symptoms of thrombosis, age at onset, family history, and contributing factors for thrombosis, and review the use of prophylactic anticoagulation in pregnant women with heterozygous type II heparin binding site defects (HBS) AT deficiency. The prevalence of thrombosis in probands with type I AT deficiency (88%) was double that observed in those with type II AT deficiency (50%)...
November 17, 2016: International Journal of Hematology
https://www.readbyqxmd.com/read/27858331/haemolysis-in-female-patient-with-chronic-phase-cml
#16
LETTER
Silvia Lovato, Robert Ayto
No abstract text is available yet for this article.
November 17, 2016: International Journal of Hematology
https://www.readbyqxmd.com/read/27858330/primary-mediastinal-large-b-cell-lymphoma-in-japanese-children-and-adolescents
#17
Tomoo Osumi, Fumiko Tanaka, Tetsuya Mori, Reiji Fukano, Masahito Tsurusawa, Koichi Oshima, Atsuko Nakazawa, Ryoji Kobayashi
This is the first case series to describe primary mediastinal large B-cell lymphoma (PMLBL) patients in children and adolescents in Asia. We retrospectively identified 17 PMLBL patients diagnosed between 1991 and 2014; in seven of these cases, the diagnosis was confirmed by central review, representing 1.0% of all NHL and 2.2% of all B-NHL cases registered. All patients were teenagers, including seven adolescents, with a median age of 14 years (range 12-18 years). Ten patients were male, and seven were female...
November 17, 2016: International Journal of Hematology
https://www.readbyqxmd.com/read/27848186/dasatinib-induces-autophagy-in-mice-with-bcr-abl-positive-leukemia
#18
Makiko Morita, Yoko Nishinaka, Itaru Kato, Satoshi Saida, Hidefumi Hiramatsu, Yasuhiko Kamikubo, Toshio Heike, Tatsutoshi Nakahata, Souichi Adachi
Dasatinib, a second-generation tyrosine kinase inhibitor, is a highly effective treatment for Bcr-Abl-positive leukemia. However, the mechanism by which dasatinib induces cell death is unclear, particularly in vivo. Autophagy is a lysosomal degradation mechanism essential for cell survival and differentiation. Autophagy also protects cells from the effects of drugs, including those used to treat leukemia. Here, we report that dasatinib induces autophagy in Bcr-Abl-positive leukemia cell lines and further show the induction of autophagy in an immunodeficient mouse model of human Bcr-Abl-positive leukemia with central nervous system (CNS) infiltration...
November 15, 2016: International Journal of Hematology
https://www.readbyqxmd.com/read/27848185/pediatric-intestinal-beh%C3%A3-et-disease-complicated-by-myeloid-malignancies
#19
Kiichiro Kanamitsu, Akira Shimada, Ritsuo Nishiuchi, Tomonari Shigemura, Yozo Nakazawa, Kenichi Koike, Yuichi Kodama, Yuichi Shinkoda, Yoshifumi Kawano, Kozo Yasui, Koji Sasaki, Ryosuke Kajiwara, Hirokazu Tsukahara, Atsushi Manabe
Behçet disease (BD) is rarely seen in children. Its clinical manifestations are believed to differ between pediatric and adult patients. The characteristics of BD complicated by myelodysplastic syndrome (MDS) are well established for adult patients; however, because only a few cases of pediatric-onset BD complicated by MDS have been reported, its clinical characteristics remain unknown. We here retrospectively review pediatric-onset BD complicated by myeloid malignancies in Japan, having identified five such patients...
November 15, 2016: International Journal of Hematology
https://www.readbyqxmd.com/read/27848184/the-initiation-and-effects-of-plasma-contact-activation-an-overview
#20
Lisha Lin, Mingyi Wu, Jinhua Zhao
The plasma contact system sits atop the intrinsic coagulation cascade and plasma kallikrein-kinin pathway, and in vivo its activation contributes, respectively, to coagulation and inflammation mainly via two downstream pathways. This system has been widely investigated, its activation mechanisms by negatively charged surfaces and the interactions within its components, factor XII, prekallikrein and high molecular weight kininogen are well understood at the biochemical level. However, as most of the activators that have been discovered by in vitro experiments are exogenous, the physiological activators and roles of the contact system have remained unclear and controversial...
November 15, 2016: International Journal of Hematology
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