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.