![]() Approval for the study was obtained from the hospital institutional research Ethics Committee (19/219-E). Data were collected conforming medical records from outpatient clinics. Follow-up time was counted as the time between patient referral to the Immunology Department and the date of last contact. Severe infection comprised sepsis, meningitis, osteomyelitis and complicated pneumonia. Infectious data were defined as: (i) recurrent upper respiratory tract infection (URTI): as greater than or equal to three episodes of rhinitis, sinusitis, otitis, pharyngitis, or tonsillitis (ii) recurrent lower respiratory tract infection (LRTI) as greater than or equal to one episode of acute bronchitis, pneumonia or community-acquired pneumonia, acute exacerbation of chronic obstructive pulmonary disease (COPD) or bronchiectasis. Definition on infection data (recurrent, severe and persistent infections, etc.) were adopted based on consensus. The diagnosis of SID was performed based on recent clinical guidelines : recurrent or severe infections, reductions of serum IgG, IgA, and/or IgM by two or more standard deviations from the normal mean, and failure to mount Ab response to polysaccharide and/or protein antigens. The diagnosis of B-CLPD was performed based on the WHO Classification of Tumours of Haematopoietic and Lymphoid Tissue. ![]() None of the patients were under active treatment at the time of the immunological assessment. All B-CLPD patients were consecutively referred to the Clinical Immunology Department from the Haematology Department after presenting with recurrent or severe infections and/or after the finding of hypogammaglobulinemia. This is a single centre retrospective observational cohort study conducted at the Hospital Clínico San Carlos of Madrid from 2015 to 2020. Trained immunity-based vaccines (TIbV) might be a promising adjuvant strategy to reduce infections in SID patients. Management of SID patients include IgRT and prophylactic antibiotics. ![]() Here we provide a comprehensive clinical and immunological overview of a real-life cohort of SID-B-CLPD patients to build on infectious risk stratification, but also on second primary neoplasia, autoimmunity, and gastrointestinal involvement. The purpose of this investigation, in a close collaborative effort with haematologists, was to ascertain whether immunological stratification of SID-B-CLPD patients, as performed in primary immunodeficiency (PID) based on the main immune branch affected (B/T/NK cells/complement/phagocytes) may be clinically relevant. The impact of B-CLPD-associated immune defects beyond B cell defects on the clinical outcomes has not been fully addressed. Nevertheless, other immune cell types and proteins, such as CD4 + and CD8 + T-cells, myeloid suppressor cells, dendritic cells (DCs), natural killer (NK) cells, and the complement system can be profoundly altered as to render the patient susceptible to increased risk of viral and opportunistic infections. 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.įrom an immunological point of view, B-CLPD course and its treatments induce a B cell defect with predominantly humoral immune deficiency. 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. 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). ![]() 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). Patients with combined immune defect had a 3.69-fold higher risk for severe infection ( p = 0.001) than those with predominantly antibody defect. B-cell defects were present in all patients. 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 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.
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