Federica Pulvirenti, Italy
Policlinico Umberto I Infective Disease and Internal MedicinePresenter of 3 Presentations
GASTRIC CANCER IS THE MOST LETHAL COMPLICATION IN CVID: DATA FROM ITALIAN PID CENTRES AND PROPOSAL FOR A NEW SCREENING ALGORITHM
Abstract
Background and Aims
CVID is a heterogeneous disorder characterized by hypogammaglobulinemia, poor vaccine responses, and defects of memory B-cells. In addition to increased susceptibility to sinopulmonary infections, autoimmunity and granulomatous disease, an increased prevalence of lymphoma and of gastric cancer (GC) has been observed. Risk factors and surveillance strategies for GC in CVID are unclear.
Methods
Overall and GC incidence rates were evaluated in 455 CVID patients regularly followed up in three Italian Centres from 1993 to 2017 and compared with age-adjusted incidence rates in the Italian Cancer Registry database.
Results
CVID patients showed an increased cancer incidence for all sites combined (Obs = 133, SIR = 2.4; 95%CI = 1.7–3.5), due to an excess of non-Hodgkin lymphoma (Obs = 33, SIR = 14.3; 95%CI = 8.4–22.6) and of GC (Obs = 25; SIR = 6.4; 95%CI = 3.2–12.5) (Fig.1). H.pylori was significantly related to GC (OR=5.3, 95%CI=1.1–24.8, p=0.042). In contrast to other reports, GC was the leading cause of death in CVID. Standardized mortality ratio indicated a 10.1-fold excess mortality in CVID with GC. CVID developed GC 15 years earlier than the normative population, but they had a similar overall survival. Only CVID diagnosed at early GC stage survived >24months (Fig.2). Stomach histology performed before cancer onset showed atrophic gastritis, intestinal metaplasia or dysplasia. CVID patients might progress rapidly to advanced cancer stage as shown by patients developing III-IV stage within 1 year from an endoscopy negative for dysplasia.
Conclusions
Based on this large case series of GC in CVID we proposed a new screening program for CVID
MALIGNANT LYMPHOMAS IN COMMON VARIABLE IMMUNODEFICIENCY: DATA FROM ITALIAN REFERRAL CARE CENTRES ON SURVEILLANCE, INCIDENCE, RISK-FACTORS AND SURVIVAL.
Abstract
Background and Aims
An increased prevalence of lymphoma has been observed in common variable immunodeficiency (CVID), the most frequently symptomatic primary immunodeficiency. Herein, we analyzed data on the prevalence of hematological, cancer risk, mortality and survival rate in a cohort of 455 Italian adult CVID patients compared to normative population. Detailed data on CVID patients diagnosed with lymphoma, histology, outcome and possible associated risk-factors were reported.
Methods
Lymphoma incidence rates were evaluated in 455 subjects with CVID enrolled in PID care centres in Rome, Padua and Naples from 1993 to 2017 and compared with incidence rates in AIRTUM database.
Results
CVID patients showed an increased incidence for non-Hodgkin lymphoma (NHL) (Obs=33, SIR=14.3; 95%CI=8.4–22.6), and Hodgkin's lymphoma (HD) (Obs=5, SIR=12.5, 95%CI=3.4-22.4) (Fig.1).The age at lymphoma diagnosis was 32.8 ± 4.6 years for HD and 52.4 ± 13.1 years for NHL. CVID-associated lymphomas were more likely to be of B-cell origin and occurred at extra-nodal sites in 30% of cases reported. T-cell lymphomas (peripheral T-cell lymphoma, angio-immunoblastic T-cell lymphoma and anaplastic T-cell lymphoma) and one primitive effusion cavity lymphomas were also observed. Patients with lymphoma were more likely to have lymphopenia (lymphocytes < 1,000 cell/mm3, OR=3.0, 95%CI=1.1–8.3, p=0.030) and polyclonal lymphocytic infiltration (OR=2.7, 95%CI=1.2–6.3, p=0.016) before cancer diagnosis.CVID patients with lymphoma had a worse survival in comparison to cancer-free CVID (HR: 4.2, 95%CI: 2.8–44.4, Fig.2). Lymphoma was the tird cause of death, following gastric cancer and respiratory infections,
Conclusions
Based on this large series, we highlighted the need of a strategy for hematological malignancy surveillance based on epidemiological studies.
HEALTH-RELATED QUALITY OF LIFE IN CVID PATIENTS RECEIVING DIFFERENT SCHEDULES OF IMMUNOGLOBULIN ADMINISTRATION: PROSPECTIVE MULTICENTER STUDY.
Abstract
Background and Aims
We assessed the health-related quality of life (HRQoL) in CVID adults receiving different schedules of immunoglobulin replacement therapy (IgRT) by intravenous (IVIG), subcutaneous (SCIG), and facilitated (fSCIG) preparations. For these patients, IgRT schedule was chosen after a period focused on identifying the most suitable individual option.
Methods
327 participants were enrolled in a prospective, observational, 18-month study. Participants received IgRT for at least 2 years. The first 6-months were devoted to the educational process during which the choices related to IgRT were regularly re-assessed, and the shift to alternative regimen was permitted. During the following 12 months, clinical data were prospectively collected, and only patients who did not further modify their IgRT schedule were included in the analysis of HRQoL measured by CVID_QoL, a specific instrument, and by GHQ-12, a tool to assess minor psychiatric nonpsychotic disorders (Fig.1).
Results
304 patients were included in the analysis. CVID_QoL global score and its dimensions (emotional functioning, relational functioning, gastrointestinal symptoms) were similar in IVIG, SCIG, and fSCIG recipients. Patients receiving IgRT by different routes of administration reported similar capacity to make long-term plans, discomfort due to therapy, and concern to run out of medications (Fig2). Multivariate analysis revealed the GHQ-12 status, but not the IgRT mode of administration, as the major factor impacting on treatment-related QoL items, and a significant impact of age on discomfort related to IgRT.
Conclusions
IgRT schedules do not impact the HRQoL in CVID if the treatment is established after an extensive educational period focused on individualizing the best therapeutic regimen