Myrthes T. Barros, Brazil

UNIVERSITY OF SÃO PAULO Clinical Immunology and Allergy

Presenter of 2 Presentations

Poster Display Malignancy and PID

PREVALENCE OF MALIGNANCIES IN COMMON VARIABLE IMMUNODEFICIENCY

Lecture Time
10:09 - 10:10
Room
Poster Area
Date
20.09.2019, Friday
Session Time
10:00 - 17:00
Board Number
10
Presentation Topic
Malignancy and PID

Abstract

Background and Aims

CVID courses with infections, autoimmunity, benign lymphoproliferations, and increased incidence of malignancies such as lymphomas and carcinomas. The most prevalent neoplasm is lymphoma followed by gastric cancer. Our objective was to evaluate the prevalence of malignancies in CVID patients from 1981 to 2019, followed at Division of Clinical Immunology and Allergy, HCFMUSP, São Paulo/Brazil.

Methods

Review of medical records of adult CVID patients.

Results

We evaluated 180 CVID patients (55% women). Mean age at onset was 13.2y, mean age at diagnosis, 33.6y. Time between onset and diagnosis was 20.3y and time of IVIG replacement was 21.4y. Thirty-five patients developed cancer: 13 gastric tumor; 6 non-Hodgkin and 1 Hodgkin lymphoma; 7 skin; 2 colorectal; 2 thyroid; 2 breast; 1 gallbladder; 1 adrenal cancer. Average disease time of patients was 36y, and mean age at cancer diagnosis 45.2y, being their mean ID diagnosis before cancer 30.8y. Regarding gastric cancer, 12 patients presented adenocarcinoma and one well differentiated neuroendocrine tumor. Five died, mean time of fatal evolution, 48.6mo. There was no age difference between gastric patients’ survivors and non-survivors. Ten patients presented gastric atrophy, autoimmunity and lymphocyte number alterations, 11 intestinal metaplasia, 7 H. pylori (+) and all had chronic diarrhea and IgA deficiency. We observed an increase in gastric tumors’ prevalence in the last 5 years, being before 2013, 0.23 new cases/year and after, 1.20 new cases/year.

Conclusions

Unlike reported in literature, the most frequent malignancy observed in our cohort was gastric cancer. We observed a striking increase in incidence in the last five years.

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Poster Display Other

55L ALLELE OF PARAOXONASE-1 MAY BE PREDICTIVE OF REDUCED MORBIDITY AND HIGHER SURVIVAL IN PATIENTS WITH COMMON VARIABLE IMMUNODEFICIENCY (CVID)

Lecture Time
10:04 - 10:05
Room
Poster Area
Date
20.09.2019, Friday
Session Time
10:00 - 17:00
Board Number
108
Presentation Topic
Other

Abstract

Background and Aims

CVID courses with infections, autoimmunity, benign lymphoproliferations, and increased incidence of malignancies, configuring a state of persistent immune activation and alterations in oxidative metabolism. There is evidence that paraoxonase-1 (PON-1), a factor controlling oxidative stress and tissue damage, is implicated in disease severity and survival. Our objective was to relate arilesterase (AA) activity, genotypes and alleles of PON1-L55M polymorphisms to disease severity and survival.

Methods

We analyzed 101 adult patients for demographic data, comorbidities, mortality and survival. PON1 genotyping (PCR-RFLP) and AA were compared to 130 healthy controls (p <0.05).

Results

Genotypic distribution was similar between groups and AA was lower in patients, which may contribute to intense inflammatory state. Patients with 55MM-genotype presented a more severe disease due to higher frequency of lymphadenopathy, splenomegaly, hepatomegaly, portal hypertension, sepsis, neoplasms and deaths compared to LM/LL-genotypes. This difference was not due to deleterious characteristic of the MM-genotype, but a protective role of the 55L allele, since patients with at least one copy of it (LM or LL) had lower prevalence of malignancies, hepatomegaly, sepsis and deaths. They also presented greater survival rates in relation to disease time and diagnostic delay. Risk of death was lower in patients with L-allele and higher in patients with chronic obstructive pulmonary disease, lymphadenopathy, neoplasms, splenomegaly, hepatomegaly and sepsis.

Conclusions

Our results suggest that 55L-allele may play a protective role in CVID, since patients with 55LL/55LM-genotypes presented lower prevalence of hepato/splenomegaly, portal hypertension, malignancies, sepsis and deaths, along with higher survival, compared to those with 55MM genotype.

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