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Acta Haematologica

Christina Schnohr, Theis Bacher, Terkel Andersen, Lars Lehrmann, Eva Funding, Lone Hvitfeldt Poulsen, Karen Binger Holm, Jakob B Bjorner
BACKGROUND: The positive effects of factor treatment of hemophilia are well established, but the long-term outcomes are not well documented. This panel study evaluated changes in bleeding frequency, joint mobility, physical function, and symptoms in Danish patients with moderate to severe hemophilia A or B over 24 years. METHODS: Three anonymous surveys were conducted in 1988, 2001, and 2012 targeting Danish patients with moderate to severe hemophilia, and the study participants, respectively, were 128, 156, and 164 male patients with hemophilia (PWH)...
November 8, 2018: Acta Haematologica
Achille Pich, Ludovica Riera, Paola Francia di Celle, Eloise Beggiato, Giulia Benevolo, Laura Godio
INTRODUCTION: Mutations in the JAK2, CALR, and MPL genes have been shown to have prognostic value in essential thrombocythaemia (ET), but no clear association with morphological changes has been reported so far. We investigated the possible correlation between gene mutations and histopathological features in bone marrow (BM) biopsies of patients with ET. METHODS: Marrow cellularity, fibrosis, and the number of total and dysmorphic megakaryocytes and clusters of megakaryocytes were compared to gene mutations in 90 cases of ET at diagnosis...
November 7, 2018: Acta Haematologica
Elisabetta Abruzzese, Malgorzata Monika Trawinska, Benedetta Neri, Francesco Bondanini, Stefano Fratoni, Andrea Tendas, Laura Scaramucci, Agostina Siniscalchi, Marco Giovannini, Roberto Palumbo, Paolo de Fabritiis, Pasquale Niscola
No abstract text is available yet for this article.
November 1, 2018: Acta Haematologica
Ertugrul Kiykim, Tanyel Zubarioglu, Mehmet Serif Cansever, Tiraje Celkan, Johannes Häberle, Ayse Cigdem Aktuglu Zeybek
BACKGROUND: Argininemia is an autosomal recessive urea cycle disorder (UCD). Unlike other UCD, hyperammonemia is rarely seen. Patients usually present in childhood with neurological symptoms. Uncommon presentations like neonatal cholestasis or cirrhosis have been reported. Although transient elevations of liver transaminases and coagulopathy have been reported during hyperammonemia episodes, a permanent coagulopathy has never been reported. METHODS: In this retrospective study, coagulation disturbances are examined in 6 argininemia patients...
October 24, 2018: Acta Haematologica
Tal Sadeh-Vered, Nurit Rosenberg, Iris Morag, Asaf A Berg, Gili Kenet, Tzipora Strauss
BACKGROUND: We evaluated the effect of surfactant on platelet function as a potential contributing mechanism to the pathogenesis of pulmonary hemorrhage (PHEM) in term and preterm infants. METHODS: Cord blood samples were collected from neonates following delivery. Complete blood count and platelet function were measured using a cone and platelet analyzer (CPA). Increasing surfactant concentrations were added to platelets in vitro, and the adhesion molecule P-selectin and the monoclonal antibody PAC-1 were evaluated following platelet activation by flow cytometry...
October 19, 2018: Acta Haematologica
Nadav Sarid, Mor Zada, Shaul Lev-Ran, Eva Yashphe, Ido Givon, Merav Barzilai, Chava Perry, Irit Avivi, Ido Wolf
Hodgkin lymphoma (HL) is one of the most curable malignancies. Despite its effectiveness, chemotherapy is often associated with adverse events (AEs) such as nausea, anorexia, and impairment of general well-being. Our objective was to assess the extent of medical cannabis use among HL patients and evaluate its efficacy in controlling chemotherapy-related AEs. Patterns of medical cannabis use and efficacy were evaluated using physician-completed application forms, medical files, and patient-completed questionnaires, for all consecutive adult HL patients treated at the Tel-Aviv Medical Center between June 2010 and November 2016...
October 19, 2018: Acta Haematologica
Thirachit Chotsampancharoen, Pornpun Sripornsawan, Sarapee Duangchu, Malai Wongchanchailert, Edward McNeil
BACKGROUND: There is scarce information on the efficacy of alternative medicine (AM) alone as a treatment for newly diagnosed acute leukemia in children. We aimed to compare overall survival (OS) between children with newly diagnosed acute leukemia who received AM alone as the first-line treatment and those treated with conventional chemotherapy (CCT). METHODS: Two-to-one nearest-neighbor propensity score-matching using sex, initial white blood cell count, phenotype of leukemia, and period of diagnosis was performed on 184 patients who received CCT and 92 who received AM alone after being diagnosed with leukemia...
October 19, 2018: Acta Haematologica
Boaz Nachmias, Adir Shaulov, Moshe E Gatt, Michael Shapira, Alexander Gural
The treatment of relapsed/refractory acute lymphoblastic leukemia (RR-ALL) presents a true clinical challenge. In 2012, a protocol combining bortezomib, dexamethasone, asparaginase, doxorubicin, and vincristine administered to children with RR-ALL was published with encouraging results. Over the past 5 years, we have implemented this protocol in the adult RR-ALL population (> 18 years) and addressed its feasibility in terms of remission rate and toxicity. Here, we present the results of our experience in 9 patients, all of whom received multiple previous chemotherapy protocols, two of them relapsing after an allogeneic bone marrow transplantation...
October 19, 2018: Acta Haematologica
Pia Raanani
No abstract text is available yet for this article.
October 18, 2018: Acta Haematologica
Lijun Jiang, Taoran Deng, Di Wang, Yi Xiao
BACKGROUND: The prognostic significance of miR-125b in intermediate-risk acute myeloid leukemia has not been well investigated. The aim of the study was to reveal the relationship between the elevated exosomal miR-125b level and the poor prognosis in adult patients with this disease. METHODS: A total of 154 consecutive patients with intermediate-risk acute myeloid leukemia were enrolled. Exosomes were isolated from blood specimens. The exosomal miR-125b level was determined using quantitative real-time polymerase chain reaction...
2018: Acta Haematologica
Fergal McGlynn, Siobhan Glavey, Jeremy Sargent, Patrick Thornton, Peter Conlon, Philip Murphy, John Quinn
No abstract text is available yet for this article.
2018: Acta Haematologica
Francesco Gaudio, Pasquale Pedote, Artor Niccoli Asabella, Giuseppe Ingravallo, Paola Sindaco, Vito Alberotanza, Tommasina Perrone, Fiomena Emanuela Laddaga, Giuseppe Rubini, Amato Antonio Stabile Ianora, Giorgina Specchia
Hodgkin's lymphoma (HL) is now a highly curable disease, with an improving 5-year survival rate that has now reached 86%. At the time of presentation, HL is usually almost entirely confined to the lymph nodes. We performed a retrospective single-institution study of 384 cases with a median follow-up of 44 months, with the aim of identifying clinical and radiological characteristics and outcomes of patients with bone HL; 32 patients (8%) had primary bone involvement, always with concurrent nodal disease. These included 22 men (69%) and 10 women (31%) with the median age as 41 years...
2018: Acta Haematologica
Constanza Martinez-Mera, Andrea Montes-Torres, Carlos Gordillo-Vélez, Javier Sánchez-Pérez
No abstract text is available yet for this article.
2018: Acta Haematologica
Anna Margret Halldorsdottir, Sveinlaug Atladottir, Margret A Thorsteinsdottir, Niels A Arnason, Gudmundur Runarsson, Torbjorn Jonsson, Olafur E Sigurjonsson, Sigrun Reykdal
BACKGROUND: Prior to infusion, cryopreserved autologous peripheral blood stem cell (auto-PBSC) grafts can either be thawed at the bedside or thawed and washed at the laboratory. At our center, manual washing of grafts prior to infusion was discontinued in April 2012 and bedside thawing was implemented. METHODS: This study compares the outcomes of two patient groups who received auto-PBSC either after post-thaw washing (n = 84) or bedside thawing (n = 83). RESULTS: No life-threatening infusion-related side effects were reported in either group...
2018: Acta Haematologica
Abdel Rahim Mahmoud Muddathir, Mozfar Idrees Abd Alla, Omar F Khabour
Cigarette smoking has been shown to be associated with changes in coagulation factors in the circulation and the subsequent thrombosis development. In this study, the impact of waterpipe smoking on the levels of fibrinogen, factor VII (FVII), and factor VIII (FVIII) was investigated. In addition, the effects of waterpipe smoking were compared to those of cigarette smoking and never smokers. A total of 80 male smokers (40 cigarette smokers and 40 waterpipe smokers) and 40 apparently healthy never smokers were recruited in the study...
2018: Acta Haematologica
Gyeong-Won Lee, Sung Woo Park, Se-Il Go, Hoon-Gu Kim, Min Kyoung Kim, Chang-Ki Min, Jae-Yong Kwak, Sang-Byung Bae, Sung-Soo Yoon, Je-Jung Lee, Ki Hwan Kim, Seung-Hyun Nam, Yeung-Chul Mun, Hyo Jung Kim, Sung Hwa Bae, Ho-Jin Shin, Jung-Hee Lee, Joon Seong Park, Seong Hyun Jeong, Mark Hong Lee, Ho Sup Lee, Keon Woo Park, Won-Sik Lee, Sang Min Lee, Jeong-Ok Lee, Myung Soo Hyun, Deog Yeon Jo, Sung-Nam Lim, Jae Hoon Lee, Hawk Kim, Do-Yeun Cho, Young Rok Do, Jeong-A Kim, Seong Kyu Park, Jin Seok Kim, Soo-Jeong Kim, Hyeon Gyu Yi, Joon Ho Moon, Chul Won Choi, Sung-Hyun Kim, Byung Soo Kim, Moo-Rim Park, Hyeok Shim, Moo-Kon Song, Youngdoe Kim, Kihyun Kim
BACKGROUND: The neutrophil-to-lymphocyte ratio (NLR) is an independent prognostic marker in solid and hematological cancers. While the derived NLR (dNLR) was shown to be non-inferior to the NLR in large cohorts of patients with different cancer types, it has not been validated as a prognostic marker for multiple myeloma (MM) to date. METHODS: Between May 22, 2011 and May 29, 2014, 176 patients with MM from 38 centers who were ineligible for autologous stem cell transplantation were analyzed...
2018: Acta Haematologica
Shao-Xue Ding, Tong Chen, Ting Wang, Chun-Yan Liu, Wen-Li Lu, Rong Fu
OBJECTIVES: This meta-analysis aimed to evaluate the risk of clonal evolution of granulocyte colony-stimulating factor (G-CSF) in acquired aplastic anemia (AA), and whether the use of G-CSF increases the occurrence of secondary malignant neoplasms, mainly myelodysplastic syndrome (MDS) and acute myeloid leukemia (AML) or paroxysmal nocturnal hemoglobinuria (PNH). METHODS: Data were gathered from randomized controlled trials (RCTs) to evaluate the effect of G-CSF versus no G-CSF at the risk of developing the clonal complications of acquired AA...
2018: Acta Haematologica
Qi Zhong, Yang-Min Zhu, Li-Ling Zheng, Hui-Juan Shen, Rui-Ming Ou, Zhi Liu, Yan-Ling She, Rui Chen, Cheng Li, Jing Huang, Meng-Dong Yao, Qing Zhang, Shuang Liu
BACKGROUND: The second-generation CD19-chimeric antigen receptor (CAR)-T co-stimulatory domain that is commonly used in clinical practice is CD28 or 4-1BB. Previous studies have shown that the persistence of CAR-T in the 4-1BB co-stimulatory domain appears to be longer. METHODS: The expression profile data of GSE65856 were obtained from GEO database. After data preprocessing, the differentially expressed genes (DEGs) between the mock CAR versus CD19-28z CAR T cells and mock CAR versus CD19-BBz CAR T cells were identified using the limma package...
2018: Acta Haematologica
Perla R Colunga-Pedraza, Gisela B Gomez-Cruz, Julia E Colunga-Pedraza, Guillermo J Ruiz-Argüelles
In 1963 Jean Bernard introduced the concept of "geographic hematology" and distinguished 2 branches, i.e., "ethnic hematology," which deals with differences between populations, and "environmental hematology," which considers factors such as food habits, infections, and others. Both of these branches have implications in the distribution of hematological diseases worldwide. In comparison with Caucasian populations, in Mexico a significantly higher prevalence of acute lymphoblastic, acute promyelocytic, and acute megakaryoblastic leukemias has been described...
2018: Acta Haematologica
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