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. 2022 Aug 19;10(8):2020.
doi: 10.3390/biomedicines10082020.

Combined Immune Defect in B-Cell Lymphoproliferative Disorders Is Associated with Severe Infection and Cancer Progression

Juliana Ochoa-Grullón  1 , Kissy Guevara-Hoyer  1 , Cristina Pérez López  2 , Rebeca Pérez de Diego  3 , Ascensión Peña Cortijo  2 , Marta Polo  2 , Marta Mateo Morales  2 , Eduardo Anguita Mandley  2 , Carlos Jiménez García  1 , Estefanía Bolaños  2 , Belén Íñigo  2 , Fiorella Medina  2 , Antonia Rodríguez de la Peña  1 , Carmen Izquierdo Delgado  1 , Eduardo de la Fuente Muñoz  1 , Elsa Mayol  1 , Miguel Fernández-Arquero  1 , Ataúlfo González-Fernández  2 , Celina Benavente Cuesta  2 , Silvia Sánchez-Ramón  1
Affiliations

Combined Immune Defect in B-Cell Lymphoproliferative Disorders Is Associated with Severe Infection and Cancer Progression

Juliana Ochoa-Grullón et al. Biomedicines. .

Abstract

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.

Keywords: B cell chronic lymphoproliferative disorders; cancer progression; combine immune defect; predominantly antibody defect; secondary immunodeficiency; severe infections.

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Conflict of interest statement

The authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest.

Figures

Figure 1
Figure 1
Proportion of clinical manifestations according to the immune defect phenotype.
Figure 2
Figure 2
Kaplan–Meier plot of progression-free and hazard ratio according to the immune defect phenotype.
Figure 3
Figure 3
Multivariate Cox’s proportional hazards regression model for cancer progression adjusted by clinical stage (low grade/high grade).
See this image and copyright information in PMC

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