Expert review of hematology
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Waldenström macroglobulinemia (WM) is a B-cell non-Hodgkin lymphoma (NHL) characterized by IgM monoclonal gammopathy and bone marrow infiltration by lymphoplasmacytic cells. Until recently, there was no drug specifically approved for WM by the US FDA, leading to wide variations in therapeutic strategies across the globe. ⋯ With identification of novel genetic mutations impacting response to ibrutinib, it would be possible to individualize therapy based on MYD88 and CXCR4 genotypes. However, long-term safety and efficacy data are required, and cost-effectiveness needs to be addressed before ibrutinib can gain widespread acceptance for front-line therapy of WM.
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Indolent non-Hodgkin's lymphoma (iNHL) describes a group of B-cell lymphomas with a long median survival and a relapsing-remitting clinical course. Although existing treatments are initially effective, patients often relapse, demonstrating decreasing efficacy with successive treatment courses. Alternative treatments are needed. ⋯ It is expressed predominantly in hematopoietic cells, making PI3Kδ an attractive therapeutic target. Idelalisib is an oral PI3Kδ inhibitor approved in 2014 in the USA and the EU as monotherapy in relapsed follicular lymphoma or relapsed small lymphocytic lymphoma previously treated with two or more prior systemic therapies, or as part of combination therapy with rituximab in patients with chronic lymphocytic leukemia, for whom rituximab monotherapy would be considered appropriate due to the presence of comorbidities. Herein, we review the available data for idelalisib, with an emphasis on relapsed/refractory B-cell iNHL.
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Correction to: Effectiveness and compliance of oral liposomal iron (Sideral Forte) treatment for iron deficiency anemia: a valid alternative to iv. iron therapies. M. Paola Campanella, Antonio Ebbli, Daniele Gaibazzi, Viviana Panunzio & Andrea Tomasini. ⋯ The revised tables and figure have been prepared (see below). Errors in data in the tables have now been corrected, and figure 2 has been corrected to amend the previously incorrect legend. The description of figure 2 has also been corrected.
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Although assessment of prior personal and familial bleeding history is an important aspect of the diagnosis of bleeding disorders, patients with mild inherited bleeding disorders are sometimes clinically asymptomatic until presented with a hemostatic challenge. However, bleeding may occur after incursion of trauma or surgery, so detection of these conditions reflects an important facet of clinical and laboratory practice. ⋯ Following determination of abnormal screening tests, subsequent investigation should follow a systematic approach that targets specific diagnostic tests, and including factor assays, full platelet function assays and more extensive specialized hemostasis testing. The current report provides a personal overview on inherited disorders of blood coagulation and their detection.
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Although Hodgkin's lymphoma is considered a highly curable cancer, a substantial portion of patients will be refractory or will relapse after first-line therapies and even after subsequent autologous stem cell transplantation. With the absence of effective salvage therapies, these patients carry a poor prognosis with dismal survival. ⋯ Such efficacy in the relapse-refractory setting has led to new hypotheses and dramatic changes in our treatment strategies of Hodgkin's lymphoma. Numerous clinical trials evaluating brentuximab in the frontline and various other treatment settings are forthcoming and will demonstrate the full extent of the drug's impact.