Ochoa Grullón, Juliana LucíaGuevara Hoyer, KissyPérez López, CristinaPérez de Diego, RebecaPeña Cortijo, AscensiónPolo, MartaMateo Morales, MartaAnguita Mandley, EduardoJiménez García, CarlosBolaños, EstefaníaÍñigo, BelénMedina, FiorellaRodríguez de la Peña, AntoniaIzquierdo Delgado, CarmenFuente Muñoz, Eduardo de laMayol, ElsaFernandez Arquero, MiguelGonzález Fernández, AtaúlfoBenavente Cuesta, CelinaSánchez Ramón, Silvia María2023-06-222023-06-222022-08-192227-905910.3390/biomedicines10082020https://hdl.handle.net/20.500.14352/72231This research received no external funding. K.G.-H is supported by The European Social Fund (ESF) through a Río Ortega Grant for Health Research Projects by the Carlos III Health Institute (ISCIII) (CM20/00098).B cell chronic lymphoproliferative diseases (B-CLPD) are associated with secondary antibody deficiency and other innate and adaptive immune defects, whose impact on infectious risk has not been systematically addressed. We performed an immunological analysis of a cohort of 83 B-CLPD patients with recurrent and/or severe infections to ascertain the clinical relevance of the immune deficiency expression. B-cell defects were present in all patients. Patients with combined immune defect had a 3.69-fold higher risk for severe infection (p = 0.001) than those with predominantly antibody defect. Interestingly, by Kaplan–Meier analysis, combined immune defect showed an earlier progression of cancer with a hazard ratio of 3.21, than predominantly antibody defect (p = 0.005). When B-CLPD were classified in low-degree, high-degree, and plasma cell dyscrasias, risk of severe disease and cancer progression significantly diverged in combined immune defect, compared with predominantly antibody defect (p = 0.001). Remarkably, an underlying primary immunodeficiency (PID) was suspected in 12 patients (14%), due to prior history of infections, autoimmune and granulomatous conditions, atypical or variegated course and compatible biological data. This first proposed SID classification might have relevant clinical implications, in terms of predicting severe infections and cancer progression, and might be applied to different B-CLPD entities.engAtribución 3.0 Españajournal articlehttps://doi.org/10.3390/biomedicines10082020https://www.mdpi.com/journal/biomedicinesopen access612.017B cell chronic lymphoproliferative disordersSevere infectionsSecondary immunodeficiencyPredominantly antibody defectCombine immune defectCancer progressionMedicinaInmunologíaOncología32 Ciencias Médicas2412 Inmunología3201.01 Oncología