The absent and vanishing spleen: Congenital asplenia and hyposplenism-two case reports

Halbertsma, F.J.; Neeleman, C.; Weemaes, C.M.R.; Deuren, Marcel van

doi:10.1111/j.1651-2227.2005.tb03082.x

Cited by 18 publications

(7 citation statements)

References 13 publications

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“…Although uncommon, such individuals have been reported. (42)(43)(44)(45)(46)(47)(48) There have also been two documented cases of father-son pairs with isolated asplenia. (49) It may be that the reason such adult cases are rare is because most often such individuals succumb to infection early in childhood and a full autopsy may not have been carried out to identify asplenia as the root cause.…”

Section: Congenital Asplenia In Humansmentioning

confidence: 95%

Development and function of the mammalian spleen

et al. 2007

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The vertebrate spleen has important functions in immunity and haematopoiesis, many of which have been well studied. In contrast, we know much less about the mechanisms governing its early embryonic development. However, as a result of work over the past decade-mostly using knockout mice--significant progress has been made in unravelling the genetic processes governing the spleen's early development. Key genetic regulators, such as Tlx1 and Pbx1, have been identified, and we know some of the early transcriptional hierarchies that control the early patterning and proliferation of the splenic primordium. In mouse and humans, asplenia can arise as a result of laterality defects, or the spleen can be absent with no other discernible abnormalities. Surprisingly, given the spleen's diverse functions, asplenic individuals suffer no major haematopoietic or immune defects apart from a susceptibility to infection with encapsulated bacteria. Recent evidence has shed light on a previously unknown role of the spleen in the development and maintenance of specific B cell populations that are involved in the initial response to infection caused by encapsulated bacteria. The lack of these populations in asplenic mice and humans may go some way to explaining this susceptibility.

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Section: Congenital Asplenia In Humansmentioning

confidence: 95%

Development and function of the mammalian spleen

et al. 2007

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“…Functional hyposplenism or functional asplenism (asplenia) is a condition in which splenic phagocytic activity is diminished or absent in an anatomically present spleen (5,7,8). The term 'functional asplenia' was coined by Pearson et al (9) in 1969, in a study of patients with sickle cell anaemia.…”

Section: Introductionmentioning

confidence: 99%

Functional hyposplenism is an important and underdiagnosed immunodeficiency condition in children

et al. 2014

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“…This accelerated course of the disease is related to reduced response to antigen, impaired phagocytosis and decreased levels of tuftsin and properdin [1,11]. Mortality after acute presentation with sepsis is 56% [1,14,15]. Other series have reported up to an 80% mortality arising from sepsis [16,17].…”

Section: Discussionmentioning

confidence: 99%

Congenital Asplenia Revealed by Streptococcus oralis septicemia: Case Report

Fane¹,

Sodqi²,

Marih³

et al. 2017

J Antimicrob Agents

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Congenital asplenia is a rare life-threatening condition, often presenting with sepsis caused by encapsulated pathogens. It may arise as part of situs abnormalities or result from an unrelated specific defect of spleen development. Isolated congenital asplenia is a very rare condition. We report a case of asplenia revealed by severe sepsis and multi-organ failure in a previously healthy 25-year-old male how had never undergone any surgical procedures. Blood cultures grew Streptococcus oralis four days after admission. Computed tomography revealed pneumonia and asplenia. The patient was finally diagnosed as Streptococcal sepsis revealing isolated congenital asplenia. Cephalosporin and levofloxacin were administered and the patient died following cardiopulmonary arrests. No family history for this condition was reported. Clinicians should pay attention to the congenital asplenia in Streptococcal disease, particularly in the event of overwhelming sepsis. In affected individuals, the use of appropriate antibiotic prophylaxis and immunisations could save lives.

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The absent and vanishing spleen: Congenital asplenia and hyposplenism-two case reports

Abstract: Based on the present observations, we define a patient as functionally asplenic--and therefore at risk for life-threatening infections-when Howell-Jolly bodies are present in the blood smear, a very small spleen is found by ultrasound, or splenic blood flow is compromised.

Cited by 18 publications

References 13 publications

Development and function of the mammalian spleen

Development and function of the mammalian spleen

Functional hyposplenism is an important and underdiagnosed immunodeficiency condition in children

Congenital Asplenia Revealed by Streptococcus oralis septicemia: Case Report

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