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International Journal of Hematology

https://read.qxmd.com/read/37735323/clinical-significance-of-human-neutrophil-antigen-1-antibodies-in-children-with-neutropenia
#1
JOURNAL ARTICLE
Satoshi Goda, Shuhei Karakawa, Satoshi Okada, Hiroshi Kawaguchi, Emi Kurita, Mitsunori Noma, Aiko Yamaoka, Mayumi Komatsu, Ayaka Yanai, Mayu Kashihara, Teruhisa Fujii, Rie Onodera, Kikuyo Taniguchi, Mika Aizawa, Masao Kobayashi
Primary autoimmune neutropenia in young children is characterized by chronic neutropenia and positivity for antibodies against human neutrophil antigens (HNAs). This study analyzed the clinical characteristics of 402 children with neutropenia to identify differences between those with and without HNA-1 antibodies (HNA1abs). HNAabs in sera were detected by granulocyte immunofluorescence testing using flow cytometry. Relative fluorescence intensity (RFI) values were used to divide patients into positive (PG, n = 302), borderline (BG, n = 34), and negative (NG, n = 66) groups...
September 21, 2023: International Journal of Hematology
https://read.qxmd.com/read/37733171/pediatric-acute-myeloid-leukemia-and-hyperleukocytosis-with-wbc-count-greater-than-50%C3%A2-%C3%A3-%C3%A2-10-9-l
#2
JOURNAL ARTICLE
Aoli Zhang, Lipeng Liu, Suyu Zong, Lixian Chang, Xiaojuan Chen, Wenyu Yang, Ye Guo, Li Zhang, Yao Zou, Yumei Chen, Yingchi Zhang, Min Ruan, Xiaofan Zhu
BACKGROUND: Acute myeloid leukemia (AML) and hyperleukocytosis have an unfavorable prognosis, but the impact of hyperleukocytosis on the prognosis of pediatric AML remains uncertain. We investigated the clinical characteristics and prognosis of pediatric AML with hyperleukocytosis, defined as WBC ≥ 50 × 109 /L. METHODS: A total of 132 patients with newly diagnosed childhood AML with hyperleukocytosis were consecutively enrolled at our center from September 2009 to August 2021 to investigate prognostic factors and clinical outcomes...
September 21, 2023: International Journal of Hematology
https://read.qxmd.com/read/37728705/impact-of-previous-anthracycline-therapy-in-patients-with-acute-myeloid-leukemia-receiving-venetoclax
#3
JOURNAL ARTICLE
Ryujiro Hara, Shinichiro Machida, Norisato Hashimoto, Daisuke Ogiya, Hidetsugu Kawai, Shohei Kawakami, Sawako Shiraiwa, Makoto Onizuka, Yoshiaki Ogawa, Hiroshi Kawada, Kiyoshi Ando
Venetoclax (VEN) combination regimens are now recognized as effective against acute myeloid leukemia (AML). However, the prognosis of patients who do not attain a composite complete response (cCR) is extremely poor, and clinical determinants of response remain unknown. Medical records of 57 patients with AML treated with VEN combination regimens from April 2021 to March 2022 at six institutions were retrospectively analyzed. The primary endpoint was cCR, complete remission, or complete remission with incomplete hematologic recovery after one cycle of VEN combination regimen...
September 20, 2023: International Journal of Hematology
https://read.qxmd.com/read/37728704/updated-guidelines-for-chronic-active-epstein-barr-virus-disease
#4
REVIEW
Jun-Ichi Kawada, Yoshinori Ito, Koichi Ohshima, Masaki Yamada, Shinsuke Kataoka, Hideki Muramatsu, Akihisa Sawada, Taizo Wada, Ken-Ichi Imadome, Ayako Arai, Keiji Iwatsuki, Shouichi Ohga, Hiroshi Kimura
Chronic active Epstein-Barr virus disease (CAEBV), formerly named chronic active Epstein-Barr virus infection, is characterized by systemic inflammation and clonal proliferation of Epstein-Barr virus (EBV)-infected T or NK cells. As CAEBV is a potentially life-threatening illness, appropriate diagnosis and therapeutic interventions are necessary for favorable clinical outcomes. Substantial evidence regarding the pathogenesis and treatment of CAEBV has been accumulated since previous guidelines for the diagnosis of CAEBV were proposed...
September 20, 2023: International Journal of Hematology
https://read.qxmd.com/read/37707761/myeloid-sarcoma-and-pathological-fracture-a-case-report-and-review-of-literature
#5
JOURNAL ARTICLE
Sho Takeyasu, Ken Morita, Seitaro Saito, Masanori Toho, Takashi Oyama, Takafumi Obo, Kazuki Taoka, Arika Shimura, Hiroaki Maki, Eisuke Shibata, Yusuke Watanabe, Fumio Suzuki, Liuzhe Zhang, Hiroshi Kobayashi, Munetoshi Hinata, Mineo Kurokawa
Myeloid sarcoma is a rare clinical entity that presents as an isolated proliferation of leukemic cells, concurrently with or at relapse of acute myeloid leukemia (AML), myelodysplastic syndromes/neoplasms (MDS), chronic myeloid leukemia (CML), and myeloproliferative neoplasm (MPN). Myeloid sarcoma disrupts the normal architecture of its surrounding tissues. When it forms in long bones, it can cause their pathological fracture. We recently experienced a rare case of MDS presenting with myeloid sarcoma in the femur that eventually resulted in its pathological fracture...
September 14, 2023: International Journal of Hematology
https://read.qxmd.com/read/37700187/leukemic-phase-mucosa-associated-lymphoid-tissue-lymphoma
#6
JOURNAL ARTICLE
Takayuki Yotsuyanagi, Naoto Tomita, Yusuke Saiki, Akiko Uchida, Yu Uemura, Yoshinori Suzuki, Tsuneaki Hirakawa, Masayuki Kato, Naoya Nakamura, Ayako Arai
A 78-year-old man presenting with leukocytosis was admitted to our hospital. The patient was asymptomatic and showed no lymphadenopathy. Peripheral blood flow cytometry revealed a leukemic-phase B-cell lymphoma with medium-to-large abnormal cells with reticulum. Positron emission tomography/computed tomography revealed abnormal uptake in the right orbit, bone marrow, and spleen. We performed immunological staining and fluorescence in situ hybridization on tissues extracted from the right orbit and bone marrow, which led to the diagnosis of mucosa-associated lymphoid tissue (MALT) lymphoma...
September 12, 2023: International Journal of Hematology
https://read.qxmd.com/read/37689812/diagnostic-and-treatment-guidelines-for-thrombotic-thrombocytopenic-purpura-ttp-in-japan-2023
#7
JOURNAL ARTICLE
Masanori Matsumoto, Yoshitaka Miyakawa, Koichi Kokame, Yasunori Ueda, Hideo Wada, Satoshi Higasa, Hideo Yagi, Yoshiyuki Ogawa, Kazuya Sakai, Toshiyuki Miyata, Eriko Morishita, Yoshihiro Fujimura
Thrombotic thrombocytopenic purpura (TTP) can rapidly become a life-threatening condition, and the importance of its appropriate diagnosis and treatment cannot be overstated. Until recently, TTP has mainly been diagnosed by clinical findings such as thrombocytopenia and hemolytic anemia. In addition to these clinical findings, however, reduced activity of a disintegrin-like and metalloprotease with thrombospondin type 1 motif 13 (ADAMTS13) below 10% has become internationally accepted as a diagnostic criterion for TTP...
September 10, 2023: International Journal of Hematology
https://read.qxmd.com/read/37672179/a-case-of-hodgkin-lymphoma-type-richter-syndrome-presenting-as-small-intestinal-perforation
#8
JOURNAL ARTICLE
Kosuke Miki, Reiki Ogasawara, Shunsuke Sugimura, Junichi Sugita, Rintaro Nozu, Keisuke Kojima, Daisuke Hidaka, Ai Shimizu, Kohei Okada, Naoki Kobayashi, Masahiro Ogasawara, Masahiro Imamura, Yoshihiro Matsuno, Shuichi Ota
Hodgkin lymphoma type of Richter syndrome (HL-type RS) is a rare disease that arises in patients with chronic lymphocytic leukemia (CLL). HL-type RS lesions can manifest in various sites and are often accompanied by related symptoms. This is the first case report to describe diagnosis of HL-type RS after emergency surgery for gastrointestinal perforation caused by the development of a HL-type RS lesion. A 47-year-old man diagnosed with CLL three years prior began treatment with ibrutinib due to worsening anemia and splenomegaly two months prior to the emergency department presentation...
September 6, 2023: International Journal of Hematology
https://read.qxmd.com/read/37668833/real-world-data-on-induction-therapy-in-patients-with-transplant-ineligible-newly-diagnosed-multiple-myeloma-retrospective-analysis-of-598-cases-from-kansai-myeloma-forum
#9
JOURNAL ARTICLE
Yuji Shimura, Hirohiko Shibayama, Aya Nakaya, Ryosuke Yamamura, Kazunori Imada, Hitomi Kaneko, Hitoshi Hanamoto, Shin-Ichi Fuchida, Hirokazu Tanaka, Satoru Kosugi, Miki Kiyota, Toshimitsu Matsui, Junya Kanda, Masato Iida, Mitsuhiro Matsuda, Nobuhiko Uoshima, Masaru Shibano, Takahiro Karasuno, Tsuneyoshi Hamada, Kensuke Ohta, Tomoki Ito, Hideo Yagi, Satoshi Yoshihara, Chihiro Shimazaki, Shosaku Nomura, Masayuki Hino, Akifumi Takaori-Kondo, Itaru Matsumura, Yuzuru Kanakura, Junya Kuroda
To investigate the real-world clinical outcomes and management of novel drug-containing therapies for newly diagnosed multiple myeloma (MM) patients, we retrospectively analyzed data on the first-line treatment for newly diagnosed transplant-ineligible MM patients from Kansai Myeloma Forum, a registry network in Japan. A total of 598 patients treated with novel drugs between March 2007 and February 2018 were analyzed. Regimens used were VD (n = 305), Rd (n = 103), VMP (n = 97), VCD (n = 71), and VRd (n = 22)...
September 5, 2023: International Journal of Hematology
https://read.qxmd.com/read/37668832/safety-pharmacokinetics-and-efficacy-of-belantamab-mafodotin-monotherapy-in-japanese-patients-with-relapsed-or-refractory-multiple-myeloma-dreamm-11
#10
JOURNAL ARTICLE
Shinsuke Iida, Kazutaka Sunami, Yuko Mishima, Taku Fujii, Hitomi Kato, Takumi Terao, Yuki Matsuzawa, Mari Matsubara, Timothy Crossman, Brandon E Kremer, Ira Gupta
Belantamab mafodotin, a B-cell maturation antigen-targeting antibody-drug conjugate (ADC), was investigated in Japanese patients with relapsed/refractory multiple myeloma in Part 1 of the phase I DREAMM-11 study. Patients who had received ≥ 2 prior lines of therapy including a proteasome inhibitor and immunomodulatory agent were eligible. Eight patients received belantamab mafodotin monotherapy at 2.5 mg/kg (n = 4) or 3.4 mg/kg (n = 4) by intravenous infusion every 3 weeks on day 1 of each cycle until disease progression or unacceptable toxicity...
September 5, 2023: International Journal of Hematology
https://read.qxmd.com/read/37660316/prediction-of-major-bleeding-events-in-1381-patients-with-essential-thrombocythemia
#11
JOURNAL ARTICLE
Ruth Stuckey, Jean-Christophe Ianotto, Marco Santoro, Anna Czyż, Manuel M Perez Encinas, María Teresa Gómez-Casares, Maria Soledad Noya Pereira, Anna Kulikowska de Nałęcz, Aleksandra Gołos, Krzysztof Lewandowski, Łukasz Szukalski, Jesús M González-Martín, Marta Anna Sobas
The goal of therapy in essential thrombocythemia (ET) is reducing thrombotic risk. No algorithm to predict hemorrhage risk exists. The impact ofanti-platelet, cytoreductive and anticoagulation therapies on risk of major bleeding (MB) was evaluated. MB events were retrospectively analyzed in 1381 ET from 10 European centers. There were 0.286 MB events/person-year. Neither the International Thrombosis Prognostic Score for thrombosis in essential thrombocythemia (IPSET-t) nor the revised IPSET-t (r-IPSET-t) was predictive for hemorrhage-free survival at 10 years (p = 0...
September 3, 2023: International Journal of Hematology
https://read.qxmd.com/read/37651058/current-challenges-in-the-prevention-and-management-of-post-thrombotic-syndrome-towards-improved-prevention
#12
REVIEW
Julie Wang, Elise Smeath, Hui Yin Lim, Harshal Nandurkar, Hong Kuan Kok, Prahlad Ho
Post-thrombotic syndrome (PTS) is a common and potentially debilitating complication of deep vein thrombosis (DVT), affecting up to 50% of DVT patients. The consequence of this chronic condition includes reduced quality of life, increased use of the healthcare system and decreased productivity. The societal impact of this condition is projected to increase, given our ageing population and increased burden of thrombotic diseases. Despite significant recent advances in our understanding of PTS, many unanswered questions remain...
August 31, 2023: International Journal of Hematology
https://read.qxmd.com/read/37581752/measurable-residual-disease-in-japanese-patients-with-relapsed-or-refractory-chronic-lymphocytic-leukemia-treated-with-venetoclax
#13
LETTER
Koji Izutsu, Kazuhito Yamamoto, Koji Kato, Takayuki Ishikawa, Noriko Fukuhara, Yasuhito Terui, Ilseung Choi, Sumiko Okubo, Natsumi Ogawa, Mizu Sakai, Yasuko Nishimura, Brenda Chyla, Yan Sun, Dai Maruyama
No abstract text is available yet for this article.
August 15, 2023: International Journal of Hematology
https://read.qxmd.com/read/37578593/aortitis-after-administration-of-pegfilgrastim-to-a-healthy-donor-for-peripheral-blood-stem-cell-collection
#14
JOURNAL ARTICLE
Yu Uemura, Kumi Oshima, Aika Fuseya, Akane Hosokai, Ayaka Ohashi, Masatoshi Kanno, Ayako Arai
A 45-year-old man who was a sibling donor for allogeneic peripheral blood stem cell transplantation (allo-PBSCT) was administered 7.2 mg of pegfilgrastim for stem cell collection. Peripheral blood stem cells were collected 4 days after administration of pegfilgrastim (Day 4) and 4.32 × 106 /kg of CD34-positive cells per recipient body weight were obtained. Fever of 38 ℃ or higher and left submandibular pain appeared on Day 6. Ultrasonography and contrast-enhanced computed tomography (CT) showed wall thickening of the carotid artery and the abdominal aorta...
August 14, 2023: International Journal of Hematology
https://read.qxmd.com/read/37561340/antibody-response-after-third-dose-of-covid-19-mrna-vaccination-in-allogeneic-hematopoietic-stem-cell-transplant-recipients-is-comparable-to-that-in-healthy-counterparts
#15
JOURNAL ARTICLE
Erina Takagi, Seitaro Terakura, Hidetsugu Fujigaki, Akinao Okamoto, Kotaro Miyao, Masashi Sawa, Takanobu Morishita, Tatsunori Goto, Yukiyasu Ozawa, Tetsuya Nishida, Nobuaki Fukushima, Kazutaka Ozeki, Ryo Hanajiri, Kuniaki Saito, Makoto Murata, Akihiro Tomita, Hitoshi Kiyoi
To determine the efficacy of SARS-CoV-2 mRNA vaccination for allogeneic hematopoietic stem cell transplantation (allo-HSCT) recipients, we measured antibody titer serially in 92 allo-HSCT patients. Among the evaluable 87 patients, median age at vaccination was 53 years (range, 18-75). The average time between allo-HSCT and vaccination was 3.3 years (range, 0.5-15.7). One month after the second dose, 70 patients (80.5%) had a positive response, whereas 17 patients (19.5%) had a negative response (< 20 U/mL)...
August 10, 2023: International Journal of Hematology
https://read.qxmd.com/read/37532827/covid-19-omicron-variant-outbreak-in-a-hematopoietic-stem-cell-transplant-unit
#16
JOURNAL ARTICLE
Andrea Gilioli, Paola Bresciani, Erica Franceschini, Andrea Messerotti, Valeria Pioli, Corrado Colasante, Francesca Bettelli, Davide Giusti, Fabio Forghieri, Monica Morselli, Elisabetta Colaci, Leonardo Potenza, William Gennari, Monica Pecorari, Roberto Marasca, Anna Candoni, Cristina Mussini, Tommaso Trenti, Patrizia Comoli, Mario Luppi, Angela Cuoghi
Recommendations and guidelines for management of SARS-COV-2 infection in hematologic patients were developed in the very difficult context of dealing with novel viral variants from one pandemic wave to another, with different susceptibility to available drugs and vaccines. Moreover, the largest SARS-COV-2 case series in patients treated for hematologic malignancies, including stem cell transplant recipients, was published before the Omicron surge, and refers mainly to Alpha and Delta viral variants. These infections had very high mortality, in a period when antivirals and monoclonal antibodies were mostly unavailable...
August 2, 2023: International Journal of Hematology
https://read.qxmd.com/read/37523079/a-case-of-neurodegenerative-langerhans-cell-histiocytosis-exhibiting-high-osteopontin-in-cerebrospinal-fluid-without-specific-radiological-abnormalities
#17
JOURNAL ARTICLE
Yuta Sakai, Yasuhiro Ikawa, Mika Takenaka, Kazuhiro Noguchi, Masaki Fukuda, Toshihiro Fujiki, Rie Kuroda, Shintaro Mase, Raita Araki, Hideaki Maeba, Ryosei Nishimura, Taizo Wada
Neurodegenerative Langerhans cell histiocytosis (ND-LCH) manifests several years after onset of LCH, with progressive neurological symptoms and characteristic brain imaging features. Although ND-LCH has a dismal neurological prognosis, distinct treatment strategies are not available owing to the unknown pathophysiology. We describe the case of a 6-year-old boy who developed left convergent strabismus four years after onset of multisystem LCH (MS-LCH). Although radiological imaging showed no abnormalities, the osteopontin level in the cerebrospinal fluid (CSF-OPN) was highly elevated without other abnormal CSF findings, leading to a diagnosis of ND-LCH...
July 31, 2023: International Journal of Hematology
https://read.qxmd.com/read/37515657/real-world-safety-profile-of-eculizumab-in-patients-with-paroxysmal-nocturnal-hemoglobinuria-atypical-hemolytic-uremic-syndrome-or-generalized-myasthenia-gravis-an-integrated-analysis-of-post-marketing-surveillance-in-japan
#18
JOURNAL ARTICLE
Jun-Ichi Nishimura, Tatsuya Kawaguchi, Shuichi Ito, Hiroyuki Murai, Akihiko Shimono, Takahisa Matsuda, Yuji Fukamizu, Hirozumi Akiyama, Hideo Hayashi, Takashi Nakano, Shoichi Maruyama
Eculizumab is a C5 inhibitor approved for the treatment of paroxysmal nocturnal hemoglobinuria (PNH), atypical hemolytic uremic syndrome (aHUS), and anti-acetylcholine receptor antibody-positive generalized myasthenia gravis (AChR + gMG) in Japan. We report integrated safety data from post-marketing surveillance in these three indications, focusing on commonly occurring adverse events (AEs) and infection-related AEs. Of 1219 patients registered, 1055 (PNH: 780; aHUS: 192; AChR + gMG: 83) had available safety data...
July 29, 2023: International Journal of Hematology
https://read.qxmd.com/read/37515656/a-retrospective-analysis-of-clinical-features-and-treatment-outcome-in-21-patients-with-immunoglobulin-m-related-light-chain-amyloidosis-in-japan-a-study-from-the-amyloidosis-research-committee
#19
JOURNAL ARTICLE
Shin-Ichi Fuchida, Mizuki Ogura, Tadao Ishida, Hiroyuki Hata, Hiroshi Handa, Nagaaki Katoh, Chiaki Nakaseko, Kazutaka Sunami, Yuta Katayama, Hironobu Nobata, Kazuiku Oshiro, Shinsuke Iida, Yoshiki Sekijima, Hironobu Naiki, Chihiro Shimazaki
We retrospectively gathered data of 21 patients (13 male and 8 female; median age 65 years) diagnosed with immunoglobulin M (IgM)-related light-chain (AL) amyloidosis in Japan to investigate characteristics of IgM-AL amyloidosis and its optimal treatment strategy. Median IgM and difference free light chain (FLC) at diagnosis were 1257 mg/dl and 34.3 mg/l, respectively. Organ involvement was observed in the heart in 7 patients (33%), kidneys in 15 (71%), and lymph nodes in 5 (24%). Initial treatments were melphalan/dexamethasone in 7 patients, bortezomib/cyclophosphamide/dexamethasone in 3, autologous stem cell transplantation in 3, rituximab/bendamustine in 1, other in 3, and none in 4...
July 29, 2023: International Journal of Hematology
https://read.qxmd.com/read/37493867/lenalidomide-treatment-of-japanese-patients-with-myelodysplastic-syndromes-with-5q-deletion-a-post-marketing-surveillance-study
#20
JOURNAL ARTICLE
Shuji Uno, Yoko Motegi, Kenichi Minehata, Yasuo Aoki
Lenalidomide was approved in Japan for the treatment of patients with myelodysplastic syndromes associated with a 5q deletion (del 5q-MDS) in August 2010. A post-marketing surveillance (PMS) study enrolled 173 patients with del 5q-MDS who started lenalidomide treatment between August 2010 and September 2011 (mean ± standard deviation [SD] age 72.4 ± 9.0 years) and observed for up to 6 cycles or 6 months. Adverse drug reactions (ADRs) and serious ADRs were reported in 78...
July 26, 2023: International Journal of Hematology
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