title: Secondary immunodeficiency in lymphoproliferative malignancies.
abstract: Secondary immunodeficiencies occur as a consequence of various diseases, including hematological malignancies, and the use of pharmacological therapies, such as immunosuppressive, anti-inflammatory, and biological drugs. Infections are the main cause of morbidity and mortality in multiple myeloma (MM) and chronic lymphocytic leukemia (CLL) patients. Recent advances in treatment have prolonged the duration of remission and the time between relapse phases in MM and CLL patients. However, managing multiple relapses and the use of salvage therapies can lead to cumulative immunosuppression and a higher risk of infections. The pathogenesis of immune deficiency secondary to lymphoproliferative malignancy is multifactorial including disease- and treatment-related factors. Supportive treatment, including early vaccination, anti-infective prophylaxis, and replacement immunoglobulin, plays a key role in preventing infections in MM and CLL. This article provides an overview of the basic immunology necessary to understand the pathogenesis of secondary immunodeficiency and the infectious complications in MM and CLL. We also discuss the evidence supporting the role of prophylactic replacement immunoglobulin treatment in patients with antibody failure secondary to MM and CLL and the indications for its use. Copyright © 2016 John Wiley & Sons, Ltd.
authors.affiliation: Department of Infectious Diseases, Sahlgrenska Academy, University of Gothenburg, Gothenburg, Sweden.
authors.forename: Vanda
authors.initials: V
authors.lastname: Friman
authors.affiliation: Translational Immunology Unit, Department of Medicine, Karolinska Institutet, Stockholm, Sweden.
authors.forename: Ola
authors.initials: O
authors.lastname: Winqvist
authors.affiliation: Department of Internal Medicine, Hematology Section, Sahlgrenska University Hospital, Gothenburg, Sweden.
authors.forename: Cecilie
authors.initials: C
authors.lastname: Blimark
authors.affiliation: German CLL Study Group, Department I of Internal Medicine, University Hospital of Cologne, Cologne, Germany.
authors.forename: Petra
authors.initials: P
authors.lastname: Langerbeins
authors.affiliation: Department of Clinical Immunology, University of Oxford, Oxford, UK.
authors.forename: Helen
authors.initials: H
authors.lastname: Chapel
authors.affiliation: Department of Clinical Immunology, University of Oxford, Oxford, UK.
authors.forename: Fatima
authors.initials: F
authors.lastname: Dhalla
mesh_terms: D005260:Female; D019337:Hematologic Neoplasms / Q000276:immunology / Q000628:therapy*; D006801:Humans; D007153:Immunologic Deficiency Syndromes / Q000209:etiology* / Q000276:immunology* / Q000517:prevention & control*; D015451:Leukemia, Lymphocytic, Chronic, B-Cell / Q000276:immunology / Q000628:therapy*; D008297:Male; D009101:Multiple Myeloma / Q000276:immunology / Q000628:therapy*
keywords: chronic lymphocytic leukemia; infections; multiple myeloma; prophylaxis; replacement immunoglobulin; secondary immunodeficiencies; supportive therapy