Silvia Sanchez-Ramon, SpainHospital Clinico San Carlos Clinical Immunology
Presenter Of 4 Presentations
COMPREHENSIVE CARE – MANAGING PID AND CANCER
PRIMARY AND SECONDARY IMMUNODEFICIENCY DISEASES IN ONCOHAEMATOLOGY: WHEN TO THINK ABOUT AND WHAT TO DO WITH THEM
Background and Aims
Immunodeficiencies (ID), in particular primary immunodeficiencies (PID), are often associated with haematological manifestations. Early diagnosis and management of the PID have significant prognostic implications. Secondary immunodeficiencies (SID) may also be induced by oncohaematological diseases and their treatments. Our aim was to define the oncohaematological warning signs of primary and secondary IDs in paediatric and adult patients.
A multidisciplinary group of six experts conducted a literature review and prepared a document based on agreements reached an in-person meeting. An external group of 44 IDs specialists from all over Spain assessed the document and were consulted regarding their level of agreement.
This document identifies the haematological and extra-haematological diseases that should prompt a suspicion of PIDs in adults and children, in both primary care and haematology and oncology specialists. Peripheral cytopenia and lymphoproliferative disorders are key diagnostic pointers. The diagnosis should be based on a detailed clinical history and physical exam, complete blood count and stratified laboratory tests according to the level of care. Patients who are candidates for immunoglobulin replacement therapy should be carefully selected, and treatment offered as soon as possible to avoid the development of complications. Finally, this document recommends procedures for monitoring these patients.
The resulting document is a useful tool for primary care physicians and oncohaematologists in the suspicion of ID. This document combines scientific evidence with the opinion of a broad panel of experts, and emphasizes the importance of an early diagnosis and treatment to avoid complications.
A SYSTEMATIC LITERATURE REVIEW ON THE DISEASE BURDEN OF SECONDARY IMMUNODEFICIENCY DISEASE (SID)
Background and Aims
Secondary immunodeficiency disease (SID) is a broad group of diseases characterized by hypogammaglobulinemia and caused by heterogenous etiologies such as hematological malignancies (HMs) and stem cell transplantation (SCT). This study summarized the scientific literature on disease burden of SID in patients with HMs or SCT.
Systematic searches were conducted through Medline, EMBASE, the Cochrane Library, and other databases to identify English-language articles from 1994 to 2018 reporting on the clinical, humanistic, and economic burden of SID due to HMs or SCT.
Of 906 unique publications, 21 (RCTs, n=6; observational studies, n=15) met eligibility criteria. In most (n=13) studies, patients received intravenous immunoglobulin replacement therapy (IGRT). Several studies indicated significant reductions in the number of serious bacterial infections (n=2), hospitalizations (n=2), and hospital lengths of stay (n=1) with IGRT. Two studies reported reduced usage of antibiotic therapy with IGRT; however, 1 study found no statistically significant difference. Only 3 studies reported on quality of life (all used SF-36 or a derivative measure). No economic studies were identified. Overall, the findings suggested some beneficial effects of IGRT on clinical outcomes and quality of life; however, disparate definitions of SID and serum IgG thresholds, infrequent reporting of statistical significance, and scarcity of clinical trial data post-1990s present potential areas for further investigation.
The paucity of available data indicates a clear unmet need for healthcare professionals to have current evidence available for assessing the efficacy and potential humanistic and economic benefits of IGRT in patients with SID.