Splenic Infarction in a White Man With Sickle Cell Trait

Diep, Bonnie; Scheirman, K; Reeves, W. Brian; Mask, D R; Eichner, E. Randy

doi:10.1097/00007611-197912000-00038

Cited by 8 publications

(4 citation statements)

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“…In our review, there were ten reported cases occurring in women, three of which occurred in high altitude environments (> 3000 m) [ 22 , 34 , 37 ]. Although there is more frequent reporting of males, the potential reasons underlying this phenomenon are manifold; for example, one cause among many may be related to more men than women historically engaging in mountain climbing and other strenuous activities at low oxygen levels [ 21 ].…”

Section: Discussionmentioning

confidence: 99%

Splenic infarction in sickle cell trait: A comprehensive systematic review of case studies

Jefferson

Sims

Umeh

et al. 2021

eJHaem

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Sickle cell trait (SCT), a commonly asymptomatic condition, has many associated clinical complications that upon presentation, can be very difficult to attribute to SCT. The effects of SCT on the spleen, for example, are not completely understood, though there have been a number of case reports detailing related complications in diverse populations. Our objective was to perform the first comprehensive case report review of splenic infarction in SCT patients to highlight the relevance of this seemingly rare condition. We conducted an extensive literature search reviewing case reports and case series of acute splenic infarctions from 1970 to 2020. This comprehensive search resulted in 54 articles with a total of 85 individuals. The ages ranged from 7 to 65, 12% were female. Individuals were of African‐American (26%), European (16%), South Asian (13%), Middle Eastern (7%), Latin American (7%), North or East African (4%), Mediterranean (4%), West African (1%), and unknown (22%) origins. Although splenic infarct in SCT patients has been associated with high altitudes, 39% of cases reporting altitude occurred below 3000 m. Among cases where HbS values were recorded, 88% occurred in individuals with HbS levels higher than 35%, suggesting that high HbS values may be a risk factor for splenic infarction. Our findings indicate that splenic infarct occurs across a wide range of demographic populations and environmental settings. While our understanding of SCT evolves, the findings here suggest that future advances in research and healthcare could benefit more from real‐time surveillance and registry initiation for various SCT outcomes such as splenic infarct.

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Section: Discussionmentioning

confidence: 99%

Splenic infarction in sickle cell trait: A comprehensive systematic review of case studies

Jefferson

Sims

Umeh

et al. 2021

eJHaem

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“…Despite autopsy, microscopic findings, and hemoglobin electrophoresis, the family physician had great difficulty believing the results. Complications of SCT including splenic syndrome in Caucasians have been described in the literature primarily due to exposure to altitude (50–55). Interestingly, the deceased reportedly previously experienced abdominal pain after working out in the gym; one can only speculate that SCT‐related splenic syndrome may have been the cause.…”

Section: Discussionmentioning

confidence: 99%

Sickle Cell Trait-Associated Deaths: A Case Series with a Review of the Literature*,†

Thogmartin

Wilson

Palma

et al. 2011

Journal of Forensic Sciences

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This study presents a series of 16 carriers of hemoglobin S (HbS) who died during various circumstances. Many of the cases were associated with mild to moderate exertion. The onset and/or duration of symptoms varied from a few minutes to several hours with many displaying a prolonged lucid interval with stable vital signs. Despite seeking medical treatment, sickle cell trait-related micro-occlusive crisis was never considered in the differential diagnosis. Several cases were associated with sudden death. In those deaths which were delayed, high anion gap and uncompensated metabolic acidosis were typical and were not heat related. Also characteristic were large increases in creatine kinase, alanine aminotransferase, and aspartate aminotransferase along with myoglobinemia. Although the antemortem diagnosis of rhabdomyolysis was made, the underlying cause was never deduced by the clinicians. The sickling found at autopsy is not always a postmortem artifact, and in the right circumstances can be diagnostic.

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“…Whether the splenic infarctions could be related to the acquired CD55 deficiency or whether the presence of sickle cell trait could by itself explain the infarctions is debatable. HbS heterozygotes (30% of black people in Central Africa 25 ) have only minimal clinical problems, and their life expectancy and frequency of hospitalization are not different from those of the normal population, 26 although various reports in the literature have described splenic infarcts in these patients 27,28 . In contrast, no splenic infarcts have been reported in the published cases of the hereditary Inab phenotype, and in PNH splenic infarction occurs only rarely.…”

Section: Discussionmentioning

confidence: 99%