Hyposplenism: A comprehensive review. Part II: Clinical manifestations, diagnosis, and management

Abstract

In the first part of this review, we described the physiological basis of splenic function and hypofunction. We also described the wide spectrum of diseases that can result in functional hyposplenism. In the second part of this review, we will be discussing the clinical picture, including complications, diagnostic methods, and management of hyposplenism.

Low immunoglobulin M memory B-cell percentage in patients with heterotaxy syndrome correlates with the risk of severe bacterial infection

“…These data support the use of memory B and IgM memory B-cell counts as indicators of splenic function. However, their clinical use in young patients with heterotaxy syndrome is still limited (21). In the present study, we found considerably lower memory B cell and IgM memory B-cell percentages in the heterotaxy patients compared with the control complex and simple CHD groups.…”

“…The current hyposplenia treatment guidelines in United States and Europe recommend long-term antibiotic prophylaxis for high-risk patients (21)(22)(23). However, the suggested treatment duration is controversial (23), ranging from 2 y to lifelong, and the antibiotic prophylaxis recommendation has often been questioned.…”

“…Spleen status can be assessed in imaging studies, but this is not effective in identifying patients at risk of infection from hyposplenism (22). This is because some heterotaxy patients may still have accessory splenic tissue with variable splenic immune function (i.e., atrial visceral situs discrepancy) (6,7), or have abnormal splenic function despite the presence of splenic tissue (e.g., LAI patients) (6,8,21). Directly measuring splenic immune function may be informative in identifying patients at risk of infection.…”

Hyposplenism: Comparison of different methods for determining splenic function

“…Although splenectomized individuals are evidently at risk for infection, there is a much larger, heterogeneous group of patients that has diminished splenic function or functional asplenia, associated with systemic disorders such as sickle cell disease (SCD), inflammatory bowel disease (IBD), and celiac disease [12,13]. In patients with homozygous sickle cell disease (HbSS), the function of the spleen is clearly diminished and a spectacular improvement in life expectancy has been observed following the introduction of a vaccination program and the administration of prophylactic antibiotics in children with HbSS [14].…”

Massive splenic infarction in a patient with pneumococcal septic shock and unknown celiac disease

“…3 We would like to emphasize that in patients with a massive bacteremia caused by encapsulated microorganisms and no obvious primary source of infection, it may be useful to investigate and test for a potential disease causing a functional hyposplenism, sometimes discovered in adulthood as for the patient we described in this report. 4 In addition, echo-color Doppler with contrast agent is a useful tool for a diagnosis and follow up of patients with SI. This test can be performed on patients' beds and it is particularly useful for non-transportable ICU patients.…”