Kirkineska L, Perifanis V, Vasiliadis T
3 Internal Medicine Department, Aristotle University of Thessaloniki, Papageorgiou Hospital.
1 Propedeutic Internal Medicine Department, Aristotle University of Thessaloniki, AHEPA Hospital, Thessaloniki, Greece.
Hippokratia. 2014 Jan;18(1):7-11.
Functional hyposplenism is a condition accompanying many diseases such as sickle cell disease, celiac disease, alcoholic liver disease, hepatic cirrhosis, lymphomas and autoimmune disorders. It is characterised mostly by defective immune responses against infectious agents, especially encapsulated organisms, since the spleen is thought to play an important role in the production and maturation of B-memory lymphocytes and other substances like opsonins, both of which are considered crucial elements of the immune system for fighting infections. It is also associated with thrombocytosis, which might lead to thromboembolic events. Functional hyposplenism is diagnosed by the presence of Howell-Jolly bodies and pitted erythrocytes in the peripheral blood smear, and by nuclear imaging modalities such as spleen scintigraphy with the use of Technetium-99m and/or spleen scintigraphy with the use of heat-damaged Technetium-99m labeled erythrocytes. Severe infections accompanying functional hyposplenism can lead to the overwhelming post infection syndrome, which can often be fatal. Identifying patients with functional hyposplenism is important because simple measures such as vaccination against common infective microorganisms (e.g. Streptococcus pneumonia, Neisseria meningitides and Haemophilous influenzae) and antibiotic therapy when needed are considered beneficial in diminishing the frequency and gravity of the infections accompanying the syndrome.
功能性脾功能减退是一种伴随多种疾病出现的病症,如镰状细胞病、乳糜泻、酒精性肝病、肝硬化、淋巴瘤和自身免疫性疾病。其主要特征是针对感染因子,尤其是有荚膜微生物的免疫反应存在缺陷,因为脾脏被认为在B记忆淋巴细胞以及诸如调理素等其他物质的产生和成熟过程中发挥着重要作用,而这两者都被视为免疫系统对抗感染的关键要素。它还与血小板增多症有关,这可能会导致血栓栓塞事件。功能性脾功能减退通过外周血涂片中有豪-焦小体和有核红细胞,以及通过核成像检查手段来诊断,如使用99m锝的脾脏闪烁扫描和/或使用热损伤的99m锝标记红细胞的脾脏闪烁扫描。功能性脾功能减退伴随的严重感染可导致感染后暴发性综合征,这通常可能是致命的。识别功能性脾功能减退患者很重要,因为诸如针对常见感染微生物(如肺炎链球菌、脑膜炎奈瑟菌和流感嗜血杆菌)进行疫苗接种以及在需要时进行抗生素治疗等简单措施,被认为有助于减少该综合征伴随感染的频率和严重程度。