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Acta Haematologica

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https://www.readbyqxmd.com/read/28614815/a-case-of-pulmonary-langerhans-cell-sarcoma-simultaneously-diagnosed-with-cutaneous-langerhans-cell-histiocytosis-studied-by-whole-exome-sequencing
#1
Si-Wook Kim, Moon Ki Choi, Hye Sook Han, Hyojin Song, Youngil Koh, Seung-Myoung Son, Ok-Jun Lee, Ji Yeoun Lee, Ki Man Lee, Ki Hyeong Lee, Jihyun Kwon
Langerhans cell histiocytosis (LCH) and Langerhans cell sarcoma (LCS) are clonal proliferations of Langerhans-type cells. Unlike in LCH, the pathophysiology and clinical course of LCS are unclear due to its rarity. Here, we report the case of a 73-year-old male patient who was diagnosed with cutaneous LCH and pulmonary LCS at the same time. Pathological review of these 2 tumors revealed similar immunohistochemical findings. However, the tumor cells in LCS had more aggressive cytological features than those in LCH...
June 15, 2017: Acta Haematologica
https://www.readbyqxmd.com/read/28614806/review-of-rosai-dorfman-disease-new-insights-into-the-pathogenesis-of-this-rare-disorder
#2
Yanan Cai, Zhangzhen Shi, Yuansong Bai
Rosai-Dorfman disease (RDD) is a rare histiocytosis typically with bilateral painless cervical lymphadenopathy. Laboratory data are nonspecific, and the presence of emperipolesis in large foamy S-100+ CD1a- histiocytes is the prominent histologic feature. The pathogenesis of RDD still remains elusive. According to published studies, we propose that RDD cells might represent intermediate recruiting monocytes with differentiation blockade. Both disturbance of homoeostasis and inherent genomic alterations could contribute to initiation of the disorder through signal transduction...
June 15, 2017: Acta Haematologica
https://www.readbyqxmd.com/read/28595168/reactivation-of-multiple-infectious-pathogens-after-hematopoietic-stem-cell-transplantation
#3
Chen Sabrina Tan
No abstract text is available yet for this article.
June 9, 2017: Acta Haematologica
https://www.readbyqxmd.com/read/28591758/are-the-polyomaviruses-bk-and-jc-associated-with-opportunistic-infections-graft-versus-host-disease-or-worse-outcomes-in-adult-patients-receiving-their-first-allogeneic-stem-cell-transplantation-with-low-dose-alemtuzumab
#4
Laila Schneidewind, Thomas Neumann, Florian Knoll, Kathrin Zimmermann, Sigrun Smola, Christian Andreas Schmidt, William Krüger
BACKGROUND: The association of polyomaviruses BK and JC with other opportunistic infections and graft-versus-host disease (GvHD) in allogeneic stem cell transplantation is controversially discussed. METHODS: We conducted a retrospective study of 64 adult patients who received their first allogeneic stem cell transplantation between March 2010 and December 2014; the follow-up time was 2 years. RESULTS: Acute leukemia was the most frequent underlying disease (45...
June 8, 2017: Acta Haematologica
https://www.readbyqxmd.com/read/28586772/clinical-features-and-recurrence-pattern-of-perianal-abscess-in-patients-with-acute-myeloid-leukemia
#5
Hung Chang, Ming-Chung Kuo, Tzung-Chih Tang, Tung-Liang Lin, Jin-Hou Wu, Yu-Shin Hung, Po-Nan Wang
INTRODUCTION: Perianal abscess may develop during neutropenia periods in patients with acute myeloid leukemia (AML). The standard of care for perianal abscess in AML is unclear. METHODS: We retrospectively collected patient data in our institute from 2009 to 2012. RESULTS: Two hundred ninety-two patients with AML were analyzed. In total, 1,051 chemotherapy sessions were administered. Twenty-three patients experienced perianal abscess. Patients with perianal abscess were younger than those without (44 vs...
June 7, 2017: Acta Haematologica
https://www.readbyqxmd.com/read/28586777/patients-with-fever-of-unknown-origin-and-splenomegaly-diagnostic-value-of-splenectomy-and-preoperative-risk-factors-suggestive-of-underlying-lymphomas
#6
Lu Zhang, Wei Zhang, Huacong Cai, Xinxin Cao, Miao Chen, Jian Li, Tienan Zhu, Minghui Duan, Shujie Wang, Bing Han, Daobin Zhou
BACKGROUND: We reviewed patients with fever of unknown origin (FUO) and splenomegaly and assessed the diagnostic value of splenectomy and measured risk factors suggestive of an underlying lymphoma. METHODS: FUO patients (n = 83) who had splenomegaly and underwent splenectomy were enrolled into this retrospective single-center study. Clinical presentations were documented and risk factors suggestive of an underlying lymphoma were tested. RESULTS: Seventy-four patients (89...
2017: Acta Haematologica
https://www.readbyqxmd.com/read/28514781/hepcidin-homeostasis-and-diseases-related-to-iron-metabolism
#7
Cadiele Oliana Reichert, Joel da Cunha, Débora Levy, Luciana Morganti Ferreira Maselli, Sérgio Paulo Bydlowski, Celso Spada
Iron is an essential metal for cell survival that is regulated by the peptide hormone hepcidin. However, its influence on certain diseases is directly related to iron metabolism or secondary to underlying diseases. Genetic alterations influence the serum hepcidin concentration, which can lead to an iron overload in tissues, as observed in haemochromatosis, in which serum hepcidin or defective hepcidin synthesis is observed. Another genetic imbalance of iron is iron-refractory anaemia, in which serum concentrations of hepcidin are increased, precluding the flow and efflux of extra- and intracellular iron...
2017: Acta Haematologica
https://www.readbyqxmd.com/read/28514780/two-distinct-bcl2-rearrangements-each-observed-in-2-independent-subclones-evolving-from-a-founder-clone-with-trisomy-12-in-a-unique-case-of-chronic-lymphocytic-leukemia-small-lymphocytic-lymphoma
#8
https://www.readbyqxmd.com/read/28514773/a-feasibility-study-of-the-full-outpatient-conduction-of-hematopoietic-transplants-in-persons-with-multiple-sclerosis-employing-autologous-non-cryopreserved-peripheral-blood-stem-cells
#9
Guillermo J Ruiz-Argüelles, Andrés A León-Peña, Mónica León-González, Ana Karen Nuñez-Cortes, Juan Carlos Olivares-Gazca, Iván Murrieta-Alvarez, Jocelyn Vargas-Espinosa, Emilio Medina-Ceballos, Yahveth Cantero-Fortiz, Alejandro Ruiz-Argüelles, Manuel A Ruiz-Delgado, Rodrigo J Ruiz-Delgado, Guillermo Ruiz-Reyes, Manuel Priesca-Marín, Merari Starlight Torres-Priego, David Blumenkron-Marroquin, Guillermo J Ruiz-Delgado
BACKGROUND: With the goal of achieving immune system reset, autologous hematopoietic stem cell transplantations have been performed in patients with multiple sclerosis (MS). MATERIAL AND METHODS: Two hundred and eighty-six consecutive patients with MS were autografted in a single center using non-frozen peripheral blood stem cells (PBSCs), on an outpatient basis and conditioning with cyclophosphamide and rituximab. The protocol was registered in ClinicalTrials.gov identifier NCT02674217...
2017: Acta Haematologica
https://www.readbyqxmd.com/read/28514772/risk-of-onset-of-hematological-malignancies-in-patients-infected-with-the-hepatitis-b-virus-results-from-a-large-scale-retrospective-cohort-study-in-china
#10
Xiaoyang Li, Yu Zheng, Hongming Zhu, Xiaojing Lin, Yunxiang Zhang, Yubing Zhao, Jiong Hu, Junmin Li
The hepatitis B virus (HBV) is a major global issue, because an increased risk of hepatocellular carcinoma among patients infected with HBV is well established. Recently, it has been suggested that HBV is associated with other human cancers. However, the association between HBV and the risk of onset of hematological malignancies remains controversial. The aim of this large-scale retrospective cohort study was to evaluate the association between HBV infection and hematological malignancies. A retrospective analysis of 86,115 newly admitted patients at Shanghai Ruijin Hospital was performed...
2017: Acta Haematologica
https://www.readbyqxmd.com/read/28478442/thrombogenesis-in-thrombophilic-pregnancy-evaluation-of-low-molecular-weight-heparin-prophylaxis
#11
Roberto Simeone, Roberta Giacomello, Germano Bruno, Sergio Parco, Natalia Maximova, Monica Martinelli, Gabriella Zito, Stefania Luppi, Gina Cervi, Giuseppe Ricci
The aim of this study is to investigate thrombogenesis and the hypercoagulable changes in pregnant women affected by thrombophilia who received low-molecular-weight heparin (LWMH) prophylaxis. We included 21 pregnant women affected by thrombophilia treated with LWMH and 20 nontreated normal pregnant women as the control group. The sample group of thrombophilic pregnant women included different conditions (factor V Leiden mutation, protein C deficiency, protein S deficiency, antiphospholipid antibodies syndrome, and combined defects)...
2017: Acta Haematologica
https://www.readbyqxmd.com/read/28472803/bortezomib-thalidomide-and-dexamethasone-vtd-induction-results-in-better-overall-survival-than-adriamycin-thalidomide-and-dexamethasone-atd-induction-in-previously-untreated-myeloma-patients-eligible-for-transplants
#12
Jia-Feng Chen, Ling-Fei Xiao, Ran Zhang, Zi-Hang Zeng, Yi-Xuan Li, Xiang-Yu Meng
No abstract text is available yet for this article.
2017: Acta Haematologica
https://www.readbyqxmd.com/read/28445872/successful-management-of-decitabine-prior-to-full-dose-idarubicin-and-cytarabine-in-the-treatment-of-refractory-recurrent-acute-myeloid-leukemia
#13
Hongyu Zhao, Li Xu, Yongjian Yang, Jianhua Shao, Ping Chen, Xuebin Dong, Linping Gu, Daqi Li
AIMS: To investigate the safety and efficacy of the triple therapy of decitabine, idarubicin, and cytarabine in the treatment of refractory or recurrent acute myeloid leukemia (R/R AML). METHODS: We conducted a single-center retrospective study in which decitabine treatment was administered prior to full-dose idarubicin and cytarabine (D-IA) for 21 R/R AML patients. RESULTS: After 1 cycle of D-IA, 10/21 (47.6%) patients experienced a complete remission (CR) and 2/21 (9...
2017: Acta Haematologica
https://www.readbyqxmd.com/read/28427046/impressive-response-to-pixantrone-after-allogeneic-transplant-in-a-multiple-relapsed-diffuse-large-b-cell-lymphoma
#14
Francesco Malaspina, Cinzia Pellegrini, Beatrice Casadei, Lisa Argnani, Pier Luigi Zinzani
Diffuse large B-cell lymphoma is the most frequent histology at diagnosis among non-Hodgkin B lymphomas and can be cured in 50-70% of cases after the first-line chemotherapy regimen. Patients who do not respond to first-line treatment can undergo numerous subsequent steps, culminating in allogeneic stem cell transplant (alloSCT). A relapse after alloSCT, however, carries an awful prognosis, seeing the demise of the patient usually in the following months. Here we present the case of a multiple relapsed patient who successfully underwent therapy with pixantrone after alloSCT, obtaining a complete remission without any considerable side effects...
2017: Acta Haematologica
https://www.readbyqxmd.com/read/28419992/extended-small-dose-platelet-transfusions-in-multitransfused-hemato-oncological-patients-a-single-center-experience
#15
Anna Gurevich-Shapiro, Sharon Tzadok, Alina Rosenberg, Aida Inbal, Michal Bar-Natan, Ofir Wolach, Pia Raanani
BACKGROUND: Refractoriness to platelet transfusion, prevalent among 15-20% of hemato-oncological patients, is associated with multitransfusions and inferior outcomes. We evaluated the effectiveness of extended slow-dose transfusion (ESDT) in increasing platelet increments in multitransfused patients. METHODS: Patients treated after the implementation of ESDT were compared with historical controls treated with standard single-donor platelet (SDP) transfusions. Cohorts of early and late recipients were assembled for comparison, i...
2017: Acta Haematologica
https://www.readbyqxmd.com/read/28399542/presence-of-the-ivs-i-6-mutated-allele-in-beta-thalassemia-major-patients-correlates-with-extramedullary-hematopoiesis-incidence
#16
Nikolaos Sousos, Despoina Adamidou, Philippos Klonizakis, Alexandra Agapidou, Stamatia Theodoridou, Georgios Spanos, Kyriakos Psarras, Evaggelia Vetsiou, Timoleon-Achilleas Vyzantiadis, Efthymia Vlachaki
Extramedullary hematopoiesis (EMH) results from the extension of hematopoietic tissue beyond the confines of the bones. Since the initiation of regular transfusion programs from an early age for all thalassemia major (ΤΜ) patients, EMH has not been considered a clinical issue anymore. The present study aims to record the prevalence of EMH in chronically transfused ΤΜ patients followed at our institution and to investigate possible risk factors associated with its occurrence. The project was designed as a retrospective, nonexperimental, descriptive, exploratory study...
2017: Acta Haematologica
https://www.readbyqxmd.com/read/28399541/extramedullary-haematopoiesis-in-patients-with-thalassaemia-major-should-we-search-for-it-regularly
#17
Paolo Ricchi
No abstract text is available yet for this article.
2017: Acta Haematologica
https://www.readbyqxmd.com/read/28399540/continuing-imatinib-treatment-for-chronic-myeloid-leukemia-patients-who-had-serious-adverse-events-at-the-onset-of-therapy
#18
Yufeng Li, Chun-Ling Wang, Banhe Din, Liang Yu, Jiabin Zhu
No abstract text is available yet for this article.
2017: Acta Haematologica
https://www.readbyqxmd.com/read/28399522/autologous-stem-cell-transplantation-in-multiple-myeloma-in-the-era-of-novel-drug-induction-a-retrospective-single-center-analysis
#19
Gabriela B Thoennissen, Dennis Görlich, Ulrike Bacher, Thomas Aufenberg, Anne-Christin Hüsken, Anna Antonia Hansmeier, Georg Evers, Jan-Henrik Mikesch, Fleur Fritz, Carsten Bokemeyer, Carsten Müller-Tidow, Matthias Stelljes, Rolf M Mesters, Utz Krug, Martin H Kropff, Nils H Thoennissen, Wolfgang E Berdel
Within this retrospective single-center study, we analyzed the survival of 320 multiple myeloma (MM) patients receiving melphalan high-dose chemotherapy (HDCT) and either single (n = 286) or tandem (n = 34) autologous stem cell transplantation (ASCT) from 1996 to 2012. Additionally, the impact of novel induction regimens was assessed. Median follow-up was 67 months, median overall survival (OS) 62 months, median progression-free survival (PFS) 33 months (95% CI 27-39), and treatment-related death (TRD) 3%. Multivariate analysis revealed age ≥60 years (p = 0...
2017: Acta Haematologica
https://www.readbyqxmd.com/read/28380473/successful-management-of-a-patient-with-autoimmune-hemorrhaphilia-due-to-anti-factor-xiii-13-antibodies-complicated-by-pulmonary-thromboembolism
#20
Yoshiyuki Ogawa, Kunio Yanagisawa, Masayoshi Souri, Masahiro Mihara, Chiaki Naito, Makiko Takizawa, Takuma Ishizaki, Takeki Mitsui, Hiroshi Handa, Tsukasa Osaki, Yoshihisa Nojima, Akitada Ichinose
Autoimmune hemophilia-like disease (hemorrhaphilia) due to anti-factor XIII (FXIII) antibodies (AH13) is a very rare, life-threatening bleeding disorder. A 77-year-old woman developed macrohematuria and a right renal pelvic hematoma. The coagulation times were not prolonged, but FXIII activity and antigen levels were severely and moderately reduced to 9 and 29% of normal values, respectively. Accordingly, the FXIII-specific activity turned out to be low. FXIII inhibitor and anti-FXIII-A subunit autoantibodies were detected by a 1:1 crossmixing test and immunoblot and immunochromatographic assays...
2017: Acta Haematologica
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