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International Journal of Hematology

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https://www.readbyqxmd.com/read/28534116/myeloid-neoplasms-with-germ-line-runx1-mutation
#1
REVIEW
Yoshihiro Hayashi, Yuka Harada, Gang Huang, Hironori Harada
Familial platelet disorder with propensity to myeloid malignancies (FPD/AML) is an autosomal dominant disorder characterized by quantitative and/or qualitative platelet defects with a tendency to develop a variety of hematological malignancies. Heterozygous germ line mutations in the RUNX1 gene are responsible genetic events for FPD/AML. Notably, about half of individuals in the family with germ line mutations in RUNX1 develop overt hematological malignancies. The latency is also relatively long as an average age at diagnosis is more than 30 years...
May 22, 2017: International Journal of Hematology
https://www.readbyqxmd.com/read/28534115/complexity-of-bone-marrow-hematopoietic-stem-cell-niche
#2
REVIEW
Noboru Asada, Shoichiro Takeishi, Paul S Frenette
Hematopoietic stem cells (HSCs) that produce a variety of hematopoietic lineage cells throughout the life reside in specialized microenvironment called "niche" in the bone marrow (BM) where they are tightly regulated. With the recent advances in experimental technologies enabling the selective deletion of molecules, various types of cells in the BM have been proposed to contribute to HSC niche activity. Among these are stromal cells closely associated with the vasculature. In this review, we provide an overview of recent advances in HSC niche research, and focus on the studies describing the functional roles of perivascular cells for HSC maintenance and mobilization...
May 22, 2017: International Journal of Hematology
https://www.readbyqxmd.com/read/28527129/efficacy-and-safety-of-plerixafor-for-the-mobilization-collection-of-peripheral-hematopoietic-stem-cells-for-autologous-transplantation-in-japanese-patients-with-multiple-myeloma
#3
Masaki Ri, Kosei Matsue, Kazutaka Sunami, Chihiro Shimazaki, Akio Hayashi, Yoshinori Sunaga, Toru Sasaki, Kenshi Suzuki
To evaluate the efficacy and safety of plerixafor for the mobilization/collection of peripheral hematopoietic stem cells (HSCs) for autologous transplantation in Japanese patients with multiple myeloma (MM). In a randomized study, patients received G-CSF (filgrastim, 400 µg/m(2)/day) for 4 days prior to the first dose of plerixafor. Starting on Day 4 evening and for up to 4 days, patients received either plerixafor (240 µg/kg/day) + G-CSF group (PG group) or G-CSF alone (G group). Daily apheresis started on Day 5 for up to 4 days, or until ≥6 × 10(6) CD34+ cells/kg were collected...
May 19, 2017: International Journal of Hematology
https://www.readbyqxmd.com/read/28523571/congenital-immature-pure-erythroid-leukemia-with-e-cadherin-expression
#4
Akihiro Tamura, Suguru Uemura, Atsuro Saito, Saki Okubo, Nanako Nino, Teppei Tahara, Takehito Yokoi, Kenji Kishimoto, Toshiaki Ishida, Daiichiro Hasegawa, Keiichiro Kawasaki, Seiji Yoshimoto, Hideto Nakao, Makiko Yoshida, Yoshiyuki Kosaka
Congenital pure erythroid leukemia is exceedingly rare and poses a diagnostic challenge. We report an atypical case of congenital pure erythroid leukemia that did not express typical erythroid markers. The patient presented with a high white blood cell count with blastic cells at birth. Although flow cytometric analyses of peripheral blood and bone marrow showed a large CD45-negative cell population, we did not identify any evidence of monoclonality. While the circulating blasts decreased with only supportive care, hepatomegaly with multiple nodules was accompanied by liver failure, disseminated intravascular coagulation, and development of hemophagocytic lymphohistiocytosis...
May 18, 2017: International Journal of Hematology
https://www.readbyqxmd.com/read/28516403/long-term-clinical-outcome-of-spinal-langerhans-cell-histiocytosis-in-children
#5
Seong Wook Lee, Hyery Kim, Jin Kyung Suh, Kyung-Nam Koh, Ho Joon Im, Hee Mang Yoon, Jong Jin Seo
Spinal involvement of Langerhans cell histiocytosis (LCH) affects morbidity, but outcomes are not well understood. We analyzed long-term outcomes following uniform treatment at a single institution. Clinical characteristics and outcomes of spinal LCH patients were retrospectively analyzed. Height ratios were calculated using the anterior height of the involved vertebral body on magnetic resonance imaging (MRI) and the expected normal vertebral height. Twenty-two (22.4%) of 98 patients diagnosed with LCH had spinal involvement...
May 17, 2017: International Journal of Hematology
https://www.readbyqxmd.com/read/28516402/effects-of-calcineurin-inhibitors-on-sodium-excretion-in-recipients-of-allogeneic-hematopoietic-stem-cell-transplantation
#6
Masuho Saburi, Sumiko Kohashi, Jun Kato, Yuya Koda, Masatoshi Sakurai, Takaaki Toyama, Taku Kikuchi, Daiki Karigane, Sayako Yuda, Yusuke Yamane, Risa Hashida, Ryohei Abe, Tomonori Nakazato, Junichi Hirahashi, Masao Ogata, Shinichiro Okamoto, Takehiko Mori
Calcineurin inhibitors (CIs) such as cyclosporine A (CSA) and tacrolimus often cause renal dysfunction, resulting in increased serum creatinine, hyperkalemia, and hyperuricemia. However, the effects of CIs on sodium excretion have not been fully elucidated. We retrospectively evaluated the effects of CI administration on sodium excretion in recipients of allogeneic hematopoietic stem cell transplantation (HSCT). Fifty consecutive recipients each of allogeneic HSCT receiving either CSA or tacrolimus (100 patients in total) with available data for weekly fractional excretion of sodium (FENa) for a 4-week period after transplantation were enrolled in this retrospective analysis...
May 17, 2017: International Journal of Hematology
https://www.readbyqxmd.com/read/28508228/relationship-between-white-blood-cell-count-elevation-and-clinical-response-after-g-csf-priming-chemotherapy-for-acute-myeloid-leukemia
#7
Daisuke Minakata, Shin-Ichiro Fujiwara, Takashi Ikeda, Yumiko Toda, Shoko Ito, Kiyomi Mashima, Kento Umino, Hirofumi Nakano, Ryoko Yamasaki, Kaoru Morita, Yasufumi Kawasaki, Miyuki Sugimoto, Chihiro Yamamoto, Masahiro Ashizawa, Kaoru Hatano, Kazuya Sato, Iekuni Oh, Ken Ohmine, Kazuo Muroi, Yoshinobu Kanda
We retrospectively analyzed the relationship between white blood cell (WBC) count elevation after priming and clinical response in 115 patients with AML (61 untreated and 54 relapsed or refractory) treated with low-dose cytarabine, aclarubicin, and G-CSF priming. Receiver operating characteristic curve analysis showed that the ratio of maximum WBC count to pretreatment WBC count (WBCratio) was most strongly associated with complete remission (CR) in previously untreated patients among several parameters we analyzed in this study; however, the prediction accuracy was not clinically significant considering the area under the curve of 0...
May 15, 2017: International Journal of Hematology
https://www.readbyqxmd.com/read/28501929/hyponatremia-associated-with-human-herpesvirus-6-hhv-6-encephalitis-after-allogeneic-hematopoietic-stem-cell-transplantation-a-presentation-different-from-hhv-6-myelitis
#8
Koichi Murakami, Sumiko Kohashi, Masatoshi Sakurai, Jun Kato, Takaaki Toyama, Yuya Koda, Yusuke Yamane, Risa Hashida, Ryohei Abe, Rie Yamazaki, Taku Kikuchi, Takayuki Shimizu, Shigeaki Suzuki, Naoki Hasegawa, Shinichiro Okamoto, Takehiko Mori
Human herpesvirus-6 (HHV-6) encephalitis and myelitis following allogeneic hematopoietic stem cell transplantation (HSCT) is frequently life-threatening. We retrospectively evaluated the clinical significance of hyponatremia in cases of HHV-6 encephalitis/myelitis. Using an institutional database and medical records, we identified and retrospectively analyzed 16 cases of HHV-6 encephalitis and/or myelitis after allogeneic HSCT. HHV-6 encephalitis and myelitis were defined as the symptoms/signs with HHV-6-DNA in the cerebrospinal fluid...
May 13, 2017: International Journal of Hematology
https://www.readbyqxmd.com/read/28497239/validation-of-the-revised-international-prognostic-scoring-system-in-patients-with-myelodysplastic-syndrome-in-japan-results-from-a-prospective-multicenter-registry
#9
Hiroshi Kawabata, Kaoru Tohyama, Akira Matsuda, Kayano Araseki, Tomoko Hata, Takahiro Suzuki, Hidekazu Kayano, Kei Shimbo, Yuji Zaike, Kensuke Usuki, Shigeru Chiba, Takayuki Ishikawa, Nobuyoshi Arima, Masaharu Nogawa, Akiko Ohta, Yasushi Miyazaki, Kinuko Mitani, Keiya Ozawa, Shunya Arai, Mineo Kurokawa, Akifumi Takaori-Kondo
The Japanese National Research Group on Idiopathic Bone Marrow Failure Syndromes has been conducting prospective registration, central review, and follow-up study for patients with aplastic anemia and myelodysplastic syndrome (MDS) since 2006. Using this database, we retrospectively analyzed the prognosis of patients with MDS. As of May 2016, 351 cases were registered in this database, 186 of which were eligible for the present study. Kaplan-Meier analysis showed that overall survival (OS) curves of the five risk categories stipulated by the revised international prognostic scoring system (IPSS-R) were reasonably separated...
May 11, 2017: International Journal of Hematology
https://www.readbyqxmd.com/read/28474291/depleted-nitric-oxide-and-prostaglandin-e2-levels-are-correlated-with-endothelial-dysfunction-in-%C3%AE-thalassemia-hbe-patients
#10
Sudarat Satitthummanid, Noppacharn Uaprasert, Smonporn Boonyaratavej Songmuang, Ponlapat Rojnuckarin, Piyaratana Tosukhowong, Pranee Sutcharitchan, Suphot Srimahachota
Mechanisms of vascular disorders in β-thalassemia/HbE patients remain poorly understood. In the present study, we aimed to determine the presence of endothelial dysfunction and its association with altered vascular mediators in this population. Forty-three β-thalassemia/HbE patients without clinically documented vascular symptoms and 43 age-sex-matched healthy controls were enrolled. Endothelial function was assessed using flow-mediated dilatation (FMD) before and after administration of nitroglycerine (NTG)...
May 4, 2017: International Journal of Hematology
https://www.readbyqxmd.com/read/28466385/allogeneic-hematopoietic-stem-cell-transplantation-for-refractory-mycosis-fungoides-mf-and-sezary-syndrome-ss
#11
Erden Atilla, Pinar Ataca Atilla, Sinem Civriz Bozdag, Meltem Kurt Yuksel, Selami Kocak Toprak, Pervin Topcuoglu, Bengu Nisa Akay, Hatice Sanli, Gunhan Gurman, Muhit Ozcan
Cutaneous T cell lymphoma is a heterogeneous group of lymphoproliferative disorders with different clinical behavior and prognosis in which malignant T cells accumulate in the skin. In the relapsed/refractory stage, treatment strategy varies depending on clinical perspective. We retrospectively evaluated advanced stage relapse or refractory mycosis fungoides and Sezary syndrome patients who underwent allogeneic hematopoietic stem cell transplantation (allo-HSCT) at our hospital. The overall response rate was 25%, while the disease progressed and relapsed after transplant in 38% of patients...
May 2, 2017: International Journal of Hematology
https://www.readbyqxmd.com/read/28444644/ponatinib-in-japanese-patients-with-philadelphia-chromosome-positive-leukemia-a-phase-1-2-study
#12
Arinobu Tojo, Taiichi Kyo, Kazuhito Yamamoto, Hirohisa Nakamae, Naoto Takahashi, Yukio Kobayashi, Tetsuzo Tauchi, Shinichiro Okamoto, Koichi Miyamura, Kiyohiko Hatake, Hiromi Iwasaki, Itaru Matsumura, Noriko Usui, Tomoki Naoe, Meera Tugnait, Narayana I Narasimhan, Stephanie Lustgarten, Heinrich Farin, Frank Haluska, Kazuma Ohyashiki
In this ongoing Phase 1/2 study (NCT01667133), we evaluated ponatinib and assessed its recommended dose in Japanese patients with chronic myeloid leukemia (CML) resistant/intolerant to dasatinib or nilotinib, or with Philadelphia chromosome-positive acute lymphoblastic leukemia (Ph(+)ALL) resistant/intolerant to ≥1 tyrosine kinase inhibitor (TKI). The primary endpoints were safety of the recommended dose (Phase 1) and major cytogenetic response (MCyR) by 12 months in chronic-phase CML (CP-CML) patients or major hematologic response (MaHR) by 6 months in patients with advanced phase disease (Phase 2)...
April 25, 2017: International Journal of Hematology
https://www.readbyqxmd.com/read/28439775/the-prognostic-significance-of-13q-deletions-of-different-sizes-in-patients-with-b-cell-chronic-lymphoproliferative-disorders-a-retrospective-study
#13
Shuhua Yi, Heng Li, Zengjun Li, Wenjie Xiong, Huimin Liu, Wei Liu, Rui Lv, Zhen Yu, Dehui Zou, Yan Xu, Gang An, Lugui Qiu
Patients with chronic lymphocytic leukemia (CLL) with 13q deletion as the sole cytogenetic abnormality usually have a favorable outcome, but the frequency of the 13q14 deletion and its impact on the outcome of other B-cell chronic lymphoproliferative disorders (BCLPDs) remain unclear. To further characterize this aberration, we investigated the prognostic significance of 13q deletion in 541 patients with BCLPDs. We performed fluorescence in situ hybridization (FISH) studies with 13q locus-specific LSI-D13S25 and LSI-RB1 probes...
April 24, 2017: International Journal of Hematology
https://www.readbyqxmd.com/read/28429312/sox11-regulates-the-pro-apoptosis-signal-pathway-and-predicts-a-favorable-prognosis-of-mantle-cell-lymphoma
#14
Wenjuan Yang, Yanying Wang, Zhen Yu, Zengjun Li, Gang An, Wei Liu, Rui Lv, Liping Ma, Shuhua Yi, Lugui Qiu
Sex-determining region Y-box 11 (SOX11) is an important diagnostic marker in mantle cell lymphoma (MCL). However, the direct oncogenic mechanisms and downstream effector pathways implicated in SOX11-driven transformation remain poorly understood. In the present study, we analyzed SOX11 expression in B-NHL, and used lentivirus-mediated RNA interference targeting SOX11 to investigate the resulting changes in cellular processes and the underlying mechanisms in MCL cell lines. We found that patients with higher SOX11 expression have superior overall survival (OS) and progression-free survival (PFS) compared to those with lower SOX11 expression...
April 20, 2017: International Journal of Hematology
https://www.readbyqxmd.com/read/28425081/perisinusoidal-bone-marrow-involvement-by-lymphoma
#15
LETTER
Stefano Licci, Marco Rosati
No abstract text is available yet for this article.
April 19, 2017: International Journal of Hematology
https://www.readbyqxmd.com/read/28421390/ofatumumab-combined-with-chlorambucil-for-previously-untreated-chronic-lymphocytic-leukemia-a-phase-i-ii-open-label-study-in-japan
#16
Kiyohiko Hatake, Michinori Ogura, Kohichi Takada, Masafumi Taniwaki, Fanghong Zhang, Taizo Fujita, Kiyoshi Ando
Elderly/comorbid patients with chronic lymphocytic leukemia (CLL) require low-toxicity treatments. Internationally, the standard treatment for such patients is chlorambucil and an anti-CD20 therapy; however, chlorambucil is not approved in Japan. The aim of the present study was to evaluate the safety, efficacy and pharmacokinetics of ofatumumab in combination with chlorambucil in Japanese patients with previously untreated CLL who were inappropriate for fludarabine-based therapy. Ten patients were enrolled and treated in this study, all of whom received at least one dose of the study drugs...
April 18, 2017: International Journal of Hematology
https://www.readbyqxmd.com/read/28409330/microrna-192-regulates-cell-proliferation-and-cell-cycle-transition-in-acute-myeloid-leukemia-via-interaction-with-ccnt2
#17
Shun Ke, Rui-Chao Li, Jun Lu, Fan-Kai Meng, Yi-Kuan Feng, Ming-Hao Fang
MicroRNAs (miRNAs) are a class of small non-coding RNAs approximately 18-22 nucleotides in length, which play an important role in malignant transformation. The roles of miR-192 as an oncogene or tumor suppressor in solid tumors have been previously reported. However, little is known about the role of miR-192 in human acute myeloid leukemia. The results of the present study indicate that miR-192 is significantly downregulated in specimens from acute myeloid leukemia patients. Functional assays demonstrated that overexpression of miR-192 in NB4 and HL-60 cells significantly inhibited cell proliferation compared with that in control cells, and induced G0/G1 cell cycle arrest, cell differentiation, and apoptosis in vitro...
April 13, 2017: International Journal of Hematology
https://www.readbyqxmd.com/read/28409329/purification-of-leukemic-blast-cells-from-blood-smears-using-laser-microdissection
#18
Hidemasa Matsuo, Shuichi Shiga, Tsuyoshi Imai, Yasuhiko Kamikubo, Tsutomu Toki, Kiminori Terui, Etsuro Ito, Souichi Adachi
In treatment of acute myeloid leukemia (AML), prognostic factors, including gene mutation and abnormal gene expression, enable risk stratification of patients. However, in the case of a small proportion of leukemic blast cells, such as AML associated with Down syndrome (AML-DS), it is not possible to examine prognostic factors precisely due to the large proportion of normal cells. Here, we present a novel method for examining prognostic factors by making a smear on a membrane slide glass from a small amount of diagnostic specimen and collecting highly pure leukemic blast cells by laser microdissection (LMD)...
April 13, 2017: International Journal of Hematology
https://www.readbyqxmd.com/read/28409328/long-term-treatment-with-bosutinib-in-a-phase-1-2-study-in-japanese-chronic-myeloid-leukemia-patients-resistant-intolerant-to-prior-tyrosine-kinase-inhibitor-treatment
#19
Naoto Takahashi, Chiaki Nakaseko, Yukio Kobayashi, Koichi Miyamura, Chiho Ono, Yuichiro Koide, Yosuke Fujii, Kazunori Ohnishi
This long-term follow-up of a completed phase 1/2 study assessed the safety and efficacy of bosutinib in Japanese Philadelphia chromosome-positive, chronic phase (CP) or advanced phase (ADV) chronic myeloid leukemia patients who were resistant/refractory or intolerant to prior tyrosine kinase inhibitor treatment. This analysis included 63 patients with a median bosutinib follow-up of 132 weeks (range 3‒372). In the CP second-line (2L) cohort, the cumulative major cytogenetic response (MCyR) and major molecular response (MMR) rates throughout the study were 73 and 53%, respectively...
April 13, 2017: International Journal of Hematology
https://www.readbyqxmd.com/read/28405919/iron-overload-may-promote-alteration-of-nk-cells-and-hematopoietic-stem-progenitor-cells-by-jnk-and-p38-pathway-in-myelodysplastic-syndromes
#20
Yanni Hua, Chaomeng Wang, Huijuan Jiang, Yihao Wang, Chunyan Liu, Lijuan Li, Hui Liu, Zonghong Shao, Rong Fu
The objective of the study was to examine levels of intracellular iron, reactive oxygen species (ROS) and the expression of JNK and p38MAPK in NK cells and hematopoietic stem/progenitor cells (HSPCs) in MDS patients, and explore potential mechanisms by which iron overload (IOL) promotes MDS progression. Thirty-four cases of MDS and six cases of AML transformed from MDS (MDS/AML) were included. HSPCs and NK cells were isolated by magnetic absorption cell sorting. We used flow cytometry to detect the levels of ROS and intracellular JNK and P38 in NK cells and HSPCs...
April 12, 2017: International Journal of Hematology
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