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Acta Haematologica

Elena Santagostino, Guy Young, Carmen Escuriola Ettingshausen, Victor Jimenez-Yuste, Manuel Carcao
The development of inhibitors against factor VIII (FVIII) concentrates represents a significant treatment complication for hemophilia. Immune tolerance induction (ITI) therapy eradicates inhibitors in 60-80% of patients, resulting in a normal FVIII response. This article, based on presentations at the 6th International Coagulation Meeting, held in Barcelona, Spain, in September 2017, provides an overview of management approaches for patients with inhibitors and briefly tabulates four cases of ITI therapy (first-line or rescue ITI therapy in pediatric and adult patients) with successful outcomes...
February 15, 2019: Acta Haematologica
Kirk D Wyatt, Lea M Coon, Dawn N Rusk, Vilmarie Rodriguez, Deepti M Warad
The development of factor VIII inhibitors remains a significant clinical challenge in the management of hemophilia A. We present a patient of mixed ethnicity with severe hemophilia A who was found to have a F8 gene hemizygous c.5815G>T mutation resulting in an Ala1939Ser substitution (Ala1920Ser in legacy nomenclature) and possible splice site change that has been reported in only 1 patient previously. He developed an inhibitor shortly after starting replacement recombinant factor VIII (Advate®; Baxalta, Bannockburn, IL, USA) and was successfully treated with immune tolerance therapy...
February 15, 2019: Acta Haematologica
Fanfan Li, Kuangyi Shu, Jie Liu, Chenfang Shen, Xiaoou Wang, Zhaohua Zhang, Minghua Jiang
No abstract text is available yet for this article.
February 15, 2019: Acta Haematologica
Klaus Tenbrock, Sylvia Lehmann, Simone Schrading, John Moran
No abstract text is available yet for this article.
February 15, 2019: Acta Haematologica
Kenichi Tahara, Hiromi Koiso, Yo-Hei Osaki, Tomomi Sekigami, Akihiko Yokohama, Takayuki Saitoh, Norifumi Tsukamoto, Hirokazu Murakami, Masahiro Abe, Hiroshi Handa
No abstract text is available yet for this article.
February 15, 2019: Acta Haematologica
Tali Siegal, Osnat Bairey
Primary central nervous system (CNS) lymphoma is an aggressive brain tumor sensitive to chemotherapy and radiotherapy. Its incidence has increased in the elderly, and they account for the majority of patients. The median survival of patients older than 70 years did not change over the last 40 years and remained in the range of 6-7 months. The definition of elderly is nonuniform, and chronological age is not the best marker of treatment tolerability or a predictor of treatment-related toxicity. Some patients who are fit can tolerate induction, consolidation, and even high-dose chemotherapy with autologous stem cell transplantation, whereas others who have multiple comorbidities with reduced renal and bone marrow function can tolerate only intermediate doses of methotrexate...
February 15, 2019: Acta Haematologica
Bella Bielorai, Moshe Leitner, Gal Goldstein, Ruty Mehrian-Shai, Luba Trakhtenbrot, Tamar Fisher, Victoria Marcu, Michal Yalon, Ginette Schiby, Ortal Barel, Nitzan Cal, Hana Golan, Amos Toren
BACKGROUND: The WHO defined myeloid and lymphoid neoplasms (MLN) with eosinophilia associated with PDGFRB, PDGFRA, FGFR1 rearrangements as a new entity in 2016. PDGFRB-rearranged MLN sensitive to imatinib were described in adult patients. We report the first pediatric patient with PDGFRB-rearranged myeloproliferative disorder associated with T-lymphoblastic lymphoma bearing the t(5; 14)(q33;q32) translocation who was successfully treated with imatinib only. Methods/Aims: Analysis of bone marrow and peripheral blood cells by fluorescent in situ hybridization identified the PDGFRB partner as CCDC88C...
February 6, 2019: Acta Haematologica
Keisuke Tanaka, Shigeo Toyota, Megumi Akiyama, Naoki Wakimoto, Yuichi Nakamura, Yuho Najima, Noriko Doki, Kazuhiko Kakihana, Aiko Igarashi, Takeshi Kobayashi, Kazuteru Ohashi, Daisuke Kudo, Atsushi Shinagawa, Hina Takano, Takayuki Fujio, Yasushi Okoshi, Mitsuo Hori, Takashi Kumagai, Tatsuya Saito, Junichi Mukae, Koh Yamamoto, Ikuyo Tsutsumi, Takuya Komeno, Chikashi Yoshida, Masahide Yamamoto, Hiroshi Kojima
We assessed the efficacy and safety of weekly cyclophosphamide-bortezomib-dexamethasone (CBD) induction prior to autologous stem cell transplantation (ASCT) in newly diagnosed Japanese patients with multiple myeloma (MM). This regimen consisted of four 28-day cycles of once-weekly oral cyclophosphamide (300 mg/m2), subcutaneous bortezomib (1.3 mg/m2), and oral dexamethasone (40 mg). Responding patients underwent stem cell collection followed by ASCT. The primary endpoint was the postinduction rate of achieving a near complete response (nCR) or better...
February 6, 2019: Acta Haematologica
Firas El Chaer, Noa G Holtzman, Edward A Sausville, Jennie Y Law, Seung Tae Lee, Vu H Duong, Maria R Baer, Rima Koka, Zeba N Singh, Nancy M Hardy, Ashkan Emadi
Adults with relapsed or refractory (R/R) B-cell acute lymphoblastic leukemia (B-ALL) treated with conventional chemotherapy have dismal outcomes. Novel immunotherapies targeting CD19, including the bispecific T-cell engager blinatumomab and chimeric antigen-receptor T (CAR-T) cells, have revolutionized the treatment of R/R B-ALL. Robust response rates to CAR-T cell therapy after blinatumomab have recently been reported, but it is unknown whether blinatumomab can be effective following failure of anti-CD19 CAR-T cell therapy...
January 29, 2019: Acta Haematologica
Iuliana Vaxman, Morie Gertz
The term amyloidosis refers to a group of disorders in which protein fibrils accumulate in certain organs, disrupt their tissue architecture, and impair the function of the effected organ. The clinical manifestations and prognosis vary widely depending on the specific type of the affected protein. Immunoglobulin light-chain (AL) amyloidosis is the most common form of systemic amyloidosis, characterized by deposition of a misfolded monoclonal light-chain that is secreted from a plasma cell clone. Demonstrating amyloid deposits in a tissue biopsy stained with Congo red is mandatory for the diagnosis...
January 16, 2019: Acta Haematologica
Maya Zlotnick, Abraham Avigdor, Elena Ribakovsky, Arnon Nagler, Meirav Kedmi
Gemcitabine-based salvage therapy is considered an effective treatment for relapsed and refractory Non-Hodgkin's lymphoma (NHL). We analyzed the outcome of 41 consecutive NHL patients treated with gemcitabine-based regimens between January 2007 and October 2015. Twenty-eight males and 13 females (median age 66.4 years) were included. The median follow-up from gemcitabine initiation was 7.3 months. Thirty patients (73%) had B-cell, and eleven (27%) had T-cell, lymphoma. All patients received a median of 2 prior regimens, of which at least 1 was anthracycline based...
January 10, 2019: Acta Haematologica
Stephen D Smith
No abstract text is available yet for this article.
January 10, 2019: Acta Haematologica
Satoko Oka, Masaharu Nohgawa
Autoimmune diseases, including autoimmune hemolytic anemia and immune thrombocytopenic purpura, have been described in patients with non-Hodgkin lymphoma (NHL) after immunochemotherapy. However, the underlying pathogenesis remains unclear. We examined NHL patients with autoimmune cytopenia and all patients were treated with rituximab-containing therapy. The present results showed reversed imbalances in helper/suppressor T-cell populations, and an immune system imbalance may have contributed to immunological abnormalities...
January 10, 2019: Acta Haematologica
Unzela Iqbal, Aiman Rehan, Manahil Akmal, Momal Jamali, Abiha Iqbal, Bilal Ahmed Khan, Khawaja Uzair Wasif, Erika Rivera, Maliha Khan
BACKGROUND: Bibliometric analyses are a tool employed by researchers and funding agencies to establish the most important areas of research in a particular field, and to determine which foci need increased research attention. Such analyses have been published in a variety of clinical specialties; however, a detailed literature search showed that no such study has been done for "myeloid neoplasms." In order to bridge this gap, we conducted a citation analysis of the 100 most influential articles on myeloid neoplasms...
January 7, 2019: Acta Haematologica
Laila Schneidewind, Thomas Neumann, Kathrin Zimmermann, Martin Weigel, Christian Andreas Schmidt, William Krüger
BK polyomavirus-associated haemorrhagic cystitis (BKHC) is a complication after allogeneic stem cell transplantation, which can occur in 5-60% of the cases. BK viruria alone can also occur in up to 100%. BKHC can lead to severe morbidity in stem cell-transplanted patients, but data about this disease is limited. Consequently, we conducted a prospective unicentric non-interventional trial on BKHC as well as BK viruria after first adult allogeneic stem cell transplantation with a follow-up time of 1 year after inpatient treatment...
January 3, 2019: Acta Haematologica
Archana M Agarwal
No abstract text is available yet for this article.
January 2, 2019: Acta Haematologica
Guillermo J Ruiz-Argüelles
No abstract text is available yet for this article.
December 5, 2018: Acta Haematologica
Yue Song, Jing-Shi Wang, Yi-Ni Wang, Zhao Wang
Haemophagocytic lymphohistiocytosis (HLH) is a rare and severe clinical syndrome that can be classified as either primary or secondary. Secondary HLH can be triggered by a variety of diseases. Pregnancy-related HLH has already been observed clinically. However, most of these cases occur during pregnancy. Considering that the periods before and after delivery have different clinical features, we presented the first case series of HLH that presented during the postpartum stage of pregnancy. From these cases, we concluded that postpartum HLH is a common form of pregnancy-related HLH...
2019: Acta Haematologica
Oren Pasvolsky, Avi Leader
No abstract text is available yet for this article.
2019: Acta Haematologica
Li Wang, Jun Xu, Xiaolong Tian, Tingting Lv, Guolin Yuan
BACKGROUND/AIMS: The aim of this work was to investigate the efficacy and predictive factors of CLAG treatment in refractory or relapsed (R/R) acute myeloid leukemia (AML) patients. METHODS: Sixty-seven R/R AML patients were enrolled in this prospective cohort study and treated by a CLAG regimen: 5 mg/m2/day cladribine (days 1-5), 2 g/m2/day cytarabine (days 1-5), and 300 μg/day filgrastim (days 0-5). The median follow-up duration was 10 months. RESULTS: A total of 57 out of 67 patients were evaluable for remission after CLAG therapy, of whom 57...
2019: Acta Haematologica
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