AUTHOR=Sánchez-Ramón Silvia , Bermúdez Arancha , González-Granado Luis Ignacio , Rodríguez-Gallego Carlos , Sastre Ana , Soler-Palacín Pere ,  the ID-Signal Onco-Haematology Group , Allende Luis , Alsina Laia , Bielsa Ana María , Calleja-Antolín Sara , Cámara Carmen , Carbone Javier , Carreras Carmen , Martín Ana De Andrés , Deyá Angela , Díaz de Heredia Cristina , Dieli-Crimi Romina , Luis Díez José , Domínguez-Pinilla Nerea , Fernández-Pereira Luis , García José Mᵃ , Gil-Herrera Juana , Gutiérrez Antonio , Jarque Isidro , Juan Manel , Lendínez Francisco , Llobet Pilar , López Mónica , Guía Ana López de la , López-Hoyos Marcos , Martín-Nalda Andrea , Martínez Mónica , Melero Josefa , Méndez-Echevarría Ana , Moral Pedro , Neth Olaf , Núñez María , Ocejo-Vinyals Gonzalo , Ochoa-Grullón Juliana , Olbrich Peter , Oña Raquel , Pérez-Encinas Manuel , Pons Jaime , Rodríguez Carmen , Sánchez Berta , Santos-Pérez Juan Luis , Elena Seoane Mᵃ , Vlagea Alexandru 

TITLE=Primary and Secondary Immunodeficiency Diseases in Oncohaematology: Warning Signs, Diagnosis, and Management

JOURNAL=Frontiers in Immunology

VOLUME=10

YEAR=2019

URL=https://www.frontiersin.org/journals/immunology/articles/10.3389/fimmu.2019.00586

DOI=10.3389/fimmu.2019.00586

ISSN=1664-3224

ABSTRACT=<p><bold>Background:</bold> Immunodeficiencies (ID), in particular primary immunodeficiencies (PID), are often associated with haematological manifestations, such as peripheral cytopenias or lymphoproliferative syndromes. Early diagnosis and management have significant prognostic implications. Secondary immunodeficiencies (SID) may also be induced by oncohaematological diseases and their treatments. Haematologists and oncologists must therefore be aware of the association between blood disorders and cancer and ID, and be prepared to offer their patients appropriate treatment without delay. Our aim was to define the warning signs of primary and secondary IDs in paediatric and adult patients with oncohaematological manifestations.</p><p><bold>Methods:</bold> A multidisciplinary group of six experts (2 haematologists, 2 immunologists, and 2 paediatricians specializing in ID) conducted a literature review and prepared a document based on agreements reached an in-person meeting. An external group of 44 IDs specialists from all over Spain assessed the document and were consulted regarding their level of agreement.</p><p><bold>Results:</bold> This document identifies the haematological and extra-haematological diseases that should prompt a suspicion of PIDs in adults and children, in both primary care and haematology and oncology departments. Cytopenia and certain lymphoproliferative disorders are key diagnostic pointers. The diagnosis must be based on a detailed clinical history, physical exploration, complete blood count and standard laboratory tests. The immunological and haematological tests included in the diagnostic process will depend on the care level. Patients who are candidates for immunoglobulin replacement therapy must be carefully selected, and treatment should be offered as soon as possible to avoid the development of complications. Finally, this document recommends procedures for monitoring these patients.</p><p><bold>Conclusions:</bold> This document combines scientific evidence with the opinion of a broad panel of experts, and emphasizes the importance of an early diagnosis and treatment to avoid complications. The resulting document is a useful tool for primary care physicians and specialists who see both adult and paediatric patients with oncohaematological diseases.</p>