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Waldenström Macroglobulinemia
Published in Dongyou Liu, Handbook of Tumor Syndromes, 2020
Waldenström macroglobulinemia (WM) was first described in 1944 by the Swedish physician Jan Waldenström involving two cases with nasal bleeding, anemia, thrombocytopenia, lymphadenopathy, and lymphoid aggregation in the bone marrow [1]. WM is considered a distinct entity classified by the World Health Organization (WHO) as a subset of a low-grade non-Hodgkin lymphoma (NHL) and characterized by the presence of IgM monoclonal gammopathy and infiltration of the bone marrow by lymphoplasmacytic lymphoma (LPL). A small proportion (<5%) of LPL secretes a non-IgM monoclonal protein, and therefore is not classified as WM [2].
Acquired Circulating Anticoagulants Other than Lupus Anticoagulants
Published in E. Nigel Harris, Thomas Exner, Graham R. V. Hughes, Ronald A. Asherson, Phospholipid-Binding Antibodies, 2020
Similar antibodies have been reported in patients with Waldenstrom’s macroglobulinemia103 and after long term therapy with chlorpromazine.104 Most of these antibodies appear to be IgM105 and possibly the high molecular weight material simply interferes with mechanisms at the interface of plasma and a contact activator. In parallel with the highly variable bleeding tendency in patients with deficiency of factor XI, antibodies against the contact factors are similarly erratic in their clinical effect. Only those associated with Waldenstrom’s macroglobulinemia appear to pose a significant risk of bleeding.103 Other cases of bleeding in association with inhibitors against factor XI have been reported, especially when an additional risk factor such as aspirin or a surgical procedure has been present.3 A characteristic finding of these IgM anti-contact factor anticoagulants is that the patients have apparently low levels of factor XI and factor XII which fail to correct on mixing with normal plasma. Usually the celite-eluate of the patient’s plasma shows normal activity while the patients’ plasmas inhibits the celite-eluate or contact product activity of normal plasma.106
Lymphocyte and plasma cell malignancies
Published in Gabriel Virella, Medical Immunology, 2019
Juan Carlos Varela, Gabriel Virella
Combinations of rituximab, a CD20 monoclonal antibody, with cyclophosphamide and dexamethasone, or with newer drugs, such as bendamustine (an alkylating agent) or bortezomib are widely used with good results. However, bortezomib administration is associated with the development of neuropathy in about 50% of patients, and its replacement by carfilzomib (a nonneurotoxic proteasome inhibitor) has been recommended. For younger patients with Waldenström macroglobulinemia, autologous stem cell transplant and rarely allogeneic transplantation can be highly efficacious.
Low number of KIR ligands in lymphoma patients favors a good rituximab-dependent NK cell response
Published in OncoImmunology, 2021
Dhon Roméo Makanga, Maxime Jullien, Gaëlle David, Nolwenn Legrand, Catherine Willem, Léa Dubreuil, Alexandre Walencik, Cyrille Touzeau, Thomas Gastinne, Benoit Tessoulin, Steven Le Gouill, Béatrice Mahé, Katia Gagne, Patrice Chevallier, Béatrice Clemenceau, Christelle Retière
From November 2019 to February 2020, 80 consecutive adult patients treated in our institution for an NHL with a rituximab-based immunochemotherapy were included after providing informed consent. Peripheral blood mononuclear cells (PBMCs) were isolated as previously described.11 Routine information, such as disease status, previous treatments received (including anterior exposure to rituximab), and CMV serology were collected from the medical file. The best response to immunochemotherapy at the end of the follow-up period (July 2020) was determined using Cheason 2007 revised criteria21 for patients with CT-measurable disease. Patients treated for a Waldenström macroglobulinemia with no CT-measurable disease were evaluated using Owen 2012 criteria.22 In both cases, complete response (CR) was defined as the disappearance of all evidence of disease, partial response (PR) as ≥ 50% regression of measurable disease, progressive disease (PD) as the appearance of any new lesion or ≥ 50% increase measurable disease, and stable disease (SD) as failure to attain CR/PR or PD. At the time of analysis, 6 patients did not have an assessment of disease response and were excluded.
Pneumocystis pneumonia in the twenty-first century: HIV-infected versus HIV-uninfected patients
Published in Expert Review of Anti-infective Therapy, 2019
Catia Cillóniz, Cristina Dominedò, Míriam J Álvarez-Martínez, Asunción Moreno, Felipe García, Antoni Torres, José M Miro
In 2014 an interesting French study on the incidence of PcP was published by Fillatre et al. [62]. The study was carried out between 1990 and 2010 and 293 cases of PcP were detected, 154 (52%) of which in HIV-uninfected patients. The main underlying conditions associated with PcP were hematological malignancies (32%), solid tumors (18%), inflammatory diseases (15%), solid-organ transplantation (12%) and vasculitis (10%) (Figure 1). The authors grouped the risk and the incidence of PcP into three categories according to the underlying diseases or conditions: Low risk (incidence <25 cases/100,000/patient-years): inflammatory diseases, Hodgkin lymphoma, and other solid tumors.Intermediate risk (incidence 25–45 cases/100,000/patient-years): Waldenström macroglobulinemia, multiple myeloma, and central nervous system cancer.High risk (incidence >45 cases/100,000/patient-years): polyarteritis nodosa, granulomatosis with polyangiitis, polymyositis/dermatopolymyositis, acute leukemia, chronic lymphocytic leukemia, and non-Hodgkin lymphoma.
CXCR4 mutations affect presentation and outcomes in patients with Waldenström macroglobulinemia: A systematic review
Published in Expert Review of Hematology, 2019
Jorge J. Castillo, David F. Moreno, Maria I. Arbelaez, Zachary R. Hunter, Steven P. Treon
Waldenstrom macroglobulinemia (WM) is an indolent and incurable lymphoma, characterized by the accumulation of malignant IgM-secreting lymphoplasmacytic cells in the bone marrow and other organs [1]. The genomic landscape of WM is defined by recurrent somatic mutations in the MYD88 gene, which have been identified in over 90% of patients with WM [2]. The most common MYD88 mutation detected is MYD88L265P. However, other non-L265P mutations have rarely been reported in WM patients [3]. The presence of MYD88L265P has diagnostic, therapeutic and prognostic implications in patients with WM [2,4,5].