RT Journal Article T1 Combined Immune Defect in B-Cell Lymphoproliferative Disorders Is Associated with Severe Infection and Cancer Progression A1 Ochoa Grullón, Juliana Lucía A1 Guevara Hoyer, Kissy A1 Pérez López, Cristina A1 Pérez de Diego, Rebeca A1 Peña Cortijo, Ascensión A1 Polo, Marta A1 Mateo Morales, Marta A1 Anguita Mandley, Eduardo A1 Jiménez García, Carlos A1 Bolaños, Estefanía A1 Íñigo, Belén A1 Medina, Fiorella A1 Rodríguez de la Peña, Antonia A1 Izquierdo Delgado, Carmen A1 Fuente Muñoz, Eduardo de la A1 Mayol, Elsa A1 Fernandez Arquero, Miguel A1 González Fernández, Ataúlfo A1 Benavente Cuesta, Celina A1 Sánchez Ramón, Silvia María AB 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. PB MDPI SN 2227-9059 YR 2022 FD 2022-08-19 LK https://hdl.handle.net/20.500.14352/72231 UL https://hdl.handle.net/20.500.14352/72231 LA eng NO This 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). DS Docta Complutense RD 21 ago 2024