Hospital Clínico San Carlos, Madrid, Spain.
Hospital U. Marqués de Valdecilla, Santander, Spain.
Front Immunol. 2019 Mar 26;10:586. doi: 10.3389/fimmu.2019.00586. eCollection 2019.
Immunodeficiencies (ID), in particular primary immunodeficiencies (PID), are often associated with haematological manifestations, such as peripheral cytopenias or lymphoproliferative syndromes. Early diagnosis and management have significant prognostic implications. Secondary immunodeficiencies (SID) may also be induced by oncohaematological diseases and their treatments. Haematologists and oncologists must therefore be aware of the association between blood disorders and cancer and ID, and be prepared to offer their patients appropriate treatment without delay. Our aim was to define the warning signs of primary and secondary IDs in paediatric and adult patients with oncohaematological manifestations. A multidisciplinary group of six experts (2 haematologists, 2 immunologists, and 2 paediatricians specializing in ID) 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 departments. Cytopenia and certain lymphoproliferative disorders are key diagnostic pointers. The diagnosis must be based on a detailed clinical history, physical exploration, complete blood count and standard laboratory tests. The immunological and haematological tests included in the diagnostic process will depend on the care level. Patients who are candidates for immunoglobulin replacement therapy must be carefully selected, and treatment should be offered as soon as possible to avoid the development of complications. Finally, this document recommends procedures for monitoring these patients. 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. The resulting document is a useful tool for primary care physicians and specialists who see both adult and paediatric patients with oncohaematological diseases.
免疫缺陷（ID），特别是原发性免疫缺陷（PID），常伴有血液学表现，如外周血细胞减少或淋巴增殖性综合征。早期诊断和管理对预后有重要意义。继发性免疫缺陷（SID）也可能由血液系统恶性肿瘤及其治疗引起。因此，血液科医生和肿瘤学家必须意识到血液系统疾病与癌症和 ID 的相关性，并准备好及时为患者提供适当的治疗。我们的目的是确定具有血液系统恶性肿瘤表现的儿科和成年患者中原发性和继发性 ID 的预警信号。由 6 名专家（2 名血液科医生、2 名免疫学家和 2 名专门研究 ID 的儿科医生）组成的多学科小组进行了文献回顾，并根据面对面会议达成的协议编写了一份文件。来自西班牙各地的 44 名 ID 专家组成的外部小组对该文件进行了评估，并就其一致程度进行了咨询。该文件确定了在初级保健以及血液科和肿瘤科应引起对 PID 怀疑的血液学和血液系统外疾病。血细胞减少和某些淋巴增殖性疾病是关键的诊断线索。诊断必须基于详细的临床病史、体格检查、全血细胞计数和标准实验室检查。诊断过程中包括的免疫学和血液学检查将取决于治疗水平。必须仔细选择适合接受免疫球蛋白替代治疗的患者，并尽快提供治疗，以避免并发症的发生。最后，该文件建议了这些患者的监测程序。该文件结合了科学证据和广泛的专家意见，强调了早期诊断和治疗的重要性，以避免并发症。最终形成的文件是初级保健医生和治疗儿科和成年血液系统恶性肿瘤患者的专家的有用工具。
