Cardiovascular
Dave Maudgil, Anthony Watkinson in The Essential Guide to the New FRCR Part 2A and Radiology Boards, 2017
Are the following statements regarding asplenia true or false? Both bronchi are hyparterial.There is an association with ASD.Transposition of the great arteries (TGA) is associated with asplenia in 70% of cases.Situs inversus or ambiguus is associated with asplenia.Asplenia has a worse prognosis than polysplenia.
Overwhelming Post-Splenectomy Infections in the Critical Care Unit
Cheston B. Cunha, Burke A. Cunha in Infectious Diseases and Antimicrobial Stewardship in Critical Care Medicine, 2020
In order to focus the treating clinician appropriately, the patient without a spleen or with an underlying condition that may produce a functional hyposplenism should have a medical alert necklace or bracelet proclaiming this fact. Additionally, since truth can have a short half-life, the patient and his or her relatives should be aware of relating this fact to the healthcare deliverer at every visit involving an acute febrile illness. Since the splenectomy may be remote in time to the illness, this is even more important. As stated, rarely congenital asplenia may present with severe infections in adulthood, so evidence to support the diagnosis of the absent spleen should be looked for in every presentation of significant febrile illness [14].
Cardiac and cardiovascular disorders
Angus Clarke, Alex Murray, Julian Sampson in Harper's Practical Genetic Counselling, 2019
Absence of the spleen, or the presence of multiple spleens, is an important point to note at autopsy in congenital heart disease, because a combination of defects involving left-sided visceral structures is seen with asplenia, and a corresponding series involving right-sided defects with polysplenia. The recurrence risk overall is probably comparable to other types of congenital heart disease, but there is an X-linked form of isomerism for which a gene (ZIC3) has been isolated. An important group of developmental defects involving laterality and isomerism has been defined, with the heart prominently involved. Total situs inversus also provides an association with dextrocardia, as in Kartagener syndrome (see Chapter 22).
A case of systemic lupus erythematosus associated auto-splenectomy presenting as invasive pneumococcal sepsis
Published in Modern Rheumatology Case Reports, 2020
Adam Scott-Charlton, Gemma Reynolds
While auto-splenectomy is a well-documented complication of several haematological conditions, mechanism of acquired asplenia in SLE patients is unclear. The literature reports approximately 10 cases of largely asymptomatic auto-splenectomy, typically amongst patients with anti-phospholipid syndrome, characterised often by retrospective diagnoses of asplenia in the context of sepsis [1–4]. Additionally, seven cases of symptomatic spontaneous splenic rupture have been reported, characterised by systemic SIRS, a surgical abdomen and haemorrhage [5–8]. In SLE, reticuloendoethlial system involvement classically presents as lymphadenopathy or splenomegaly, but it is likely auto-splenectomy and spontaneous splenic rupture exist on a continuum of pathological splenic involvement [9]. Possible mechanisms include arterial thrombotic ischaemia secondary to antiphospholipid syndrome, organ vasculitis with silent ischaemia due to immune-complex mediated congestion of red-pulp, or lymphocyte depletion caused by circulating lymphocytotoxin [1]. Whether these mechanisms independently correlate with a specific clinical course is yet to be established due to the rarity of end-organ splenic complications and the inherent difficulties of histopathological diagnosis in a retrospective diagnosis of asplenia in the context of sepsis.
Strategies to increase access to basic sickle cell disease care in low- and middle-income countries
Published in Expert Review of Hematology, 2022
Meghna Dua, Halima Bello-Manga, Yvonne M. Carroll, Aisha Amal Galadanci, Umma Abdulsalam Ibrahim, Allison A. King, Ayobami Olanrewaju, Jeremie H. Estepp
SCD is a monogenic red blood cell (RBC) disorder, where normal hemoglobin (HbA) is replaced by sickle hemoglobin (HbS). It is inherited as an autosomal codominant trait [1], and common types of SCD include homozygous hemoglobin SS disease, hemoglobin SC disease, and sickle beta-thalassemia[1]. It is a chronic and debilitating condition characterized by hemolytic anemia and endothelial dysfunction, with findings of vaso-occlusive crises, acute chest syndrome, increased risk of stroke, and cumulative multiorgan damage. Children with SCD are also at heightened risk of morbidity and mortality from specific infections due to functional asplenia. There are approximately 300,000–400,000 babies born with SCD globally, with over 75% of them born in Africa [1]. Nigeria, India, and the Democratic Republic of the Congo alone account for over 50% of patients with SCD [2]. This geographic distribution is attributed to the ‘malaria hypothesis’ that the HbS carrier state is protective against malaria infection, which was substantiated by the coexistence of high HbS carrier rates and malaria infections in Africa [3].
Contemporary management of essential thrombocythemia in children
Published in Expert Review of Hematology, 2019
Maria Luigia Randi, Irene Bertozzi, Maria Caterina Putti
A detailed familial and personal history (headache, bleeding, paresthesia, itching, abdominal pain) must be collected, giving attention to the blood counts of relatives, and their occurrence of thrombotic event. Careful search for infections, inflammation, iron deficiency and other causes of ST is the first step of the clinical and hematological approach. An abdominal ultrasonography is useful to recognize asplenia and determine spleen size. In case of negative findings, observation of about 6 months is warranted since some cases resolve spontaneously, as suggested by the AIEOP group (http://www.aieop.org/web/). However, we are unable to define the right moment to exclude ST: in our series, in a girl presenting with extreme thrombocytosis at the age of seven, a spontaneous normalization of the platelet count occurred when she turned 15.
Related Knowledge Centers
- Birth Defect
- Polysplenia
- Spleen
- Splenectomy
- Neoplasm
- Situs Ambiguus
- Isolated Congenital Asplenia
- Splenic Injury
- Injury
- Immune Thrombocytopenic Purpura