A Case of Autosplenectomy in Sickle Cell Trait Following an Exposure to High Altitude

Yanamandra, Uday; Das, Reena; Malhotra, Pankaj; Varma, Subhash

doi:10.1016/j.wem.2017.08.021

Cited by 5 publications

(6 citation statements)

References 28 publications

(39 reference statements)

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“…As outlined in Tables 2 and 3 , the geographic location for the onset of the splenic infarction was examined for 85 cases [ 9 , 11 ‐ 23 , 25 ‐ 41 , 43 ‐ 52 , 54 ‐ 61 ]. Thirty‐two (38%) cases occurred in the United States and 41 (48%) occurred internationally (Table 2 ).…”

Section: Resultsmentioning

confidence: 99%

“…Altitude levels were reported for 59 individuals [ 9 , 11 , 13 , 17 ‐ 23 , 26 ‐ 28 , 30 , 34 , 37 , 39 , 41 , 43 , 44 , 46 ‐ 48 , 50 ‐ 52 , 55 , 57 , 59 ‐ 61 ]. Of the 59 cases reporting altitude, 2 (3%) cases occurred under 1000 m, 4 (7%) cases occurred between 1001 and 2000 m, 17 (29%) cases occurred between 2001 and 3000 m, 27 (46%) cases occurred between 3001 and 4000 m, 2 (3%) cases occurred above 4000 m, and 7 (12%) cases contained a range or ambiguous altitude levels.…”

Section: Resultsmentioning

confidence: 99%

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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: Resultsmentioning

confidence: 99%

Section: Resultsmentioning

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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“…8 Similarly, there have been reports of hypoxia-induced sickle cell crises caused by exposure to high altitudes. 9,10 When a sickle cell crisis occurs, the organs most often involved are the brain, the kidneys, the bones, the spleen, the retina, the lungs and the male external genitalia. 7 Abdominal pain crises are one of most common reasons for seeking medical attention among SCD patients, occurring in up to 50% of them.…”

Section: Discussionmentioning

confidence: 99%

Sickle cell trait: a cause of abdominal pain and pulmonary embolism

Saad

Barra

Monzoni

et al. 2020

Rev Fac Cien Med Univ Nac Cordoba

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Introduction: Sickle cell trait (SCT) is a rare and underdiagnosed disorder in the Argentinian population. In this condition, individuals carry the mutation of the HbS gene in one of the two beta-globin genes. In general, SCT does not present with the typical manifestations of sickle cell anemia. However, under certain circumstances, some clinical characteristics of the disease may develop. Methods: We discussed the case of a 39-Year old man who presented with persistent abdominal pain of unknown origin after traveling to a high-altitude place. He underwent laparotomy without a definite diagnosis. After that, the patient developed signs of splenic infarction and pulmonary thromboembolism that were confirmed by computed tomography. Results: A sickling test was positive, and a hemoglobin electrophoresis revealed an abnormal fraction at the HbS level. In this context a diagnosis of SCT was made. Additional, tests revealed a strongly positive lupus anticoagulant. Conclusion: SCT presentation as abdominal pain and thromboembolic disease in adult patients after exposure to high altitudes is a rarely suspected diagnosis.

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“…Patients with SCT present symptoms only in extreme conditions of high altitude, severe dehydration, or highintensity physical activity, [10][11][12] which is explained by red blood cell sickling and increased mechanical fragility due to the polymerization of hemoglobin HbS 13,14 with consequent microvascular occlusion resulting in complications or clinical conditions that can be lifethreatening. 7,15,16 Symptoms are common with exposure to low oxygen tension or altitude, including nonpressurized aircraft cabins, or in people exercising in mountains or the military in high-altitude locations. 7,16 When observing complications or manifestations, patients with SCT may present with splenic infarction, 6,9 venous thromboembolism (PE and DVT), 1,17 renal involvement (including proteinuria, hematuria, and chronic kidney disease), 1,18 rhabdomyolysis,19,20 sudden death, 21 and others; in paraclinical data, increased levels of bilirubin, lactate dehydrogenase (LDH), and increased reticulocyte count have been described, and on some occasions, anemia has been observed.…”

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confidence: 99%

“…7,15,16 Symptoms are common with exposure to low oxygen tension or altitude, including nonpressurized aircraft cabins, or in people exercising in mountains or the military in high-altitude locations. 7,16 When observing complications or manifestations, patients with SCT may present with splenic infarction, 6,9 venous thromboembolism (PE and DVT), 1,17 renal involvement (including proteinuria, hematuria, and chronic kidney disease), 1,18 rhabdomyolysis,19,20 sudden death, 21 and others; in paraclinical data, increased levels of bilirubin, lactate dehydrogenase (LDH), and increased reticulocyte count have been described, and on some occasions, anemia has been observed. 18,22,23 Diagnostic confirmation of SCT is performed by hemoglobin electrophoresis in gel or capillary electrophoresis or high-performance liquid chromatography (HPLC).…”

mentioning

confidence: 99%

Sickle Cell Trait, Clinical Manifestations and Outcomes: A Cross-Sectional Study

Vargas-Hernández¹,

Uscategui-Ruiz²,

Prada-Rueda³

et al. 2023

Mediterr J Hematol Infect Dis

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Introduction: Sickle cell trait (SCT) is an autosomal recessive blood disorder in which patients are heterozygous carriers for hemoglobin S (HbAS) and are usually asymptomatic. We performed a descriptive analysis of clinical manifestations and outcomes associated with SCT. Methods: This was a descriptive, cross-sectional study that included patients with SCT from 2014 to 2020 at Hospital Militar Central, the reference center of the Military forces in Bogota, Colombia. Results: Of 647 hemoglobin electrophoresis analyzed, we identified 51 patients with SCT, including 43 males (84.3%) and eight females (15.7%), with a median age of 22 years (IQR 15–36 years). Of these, 28 (54.8%) were Afro-Colombian, and 23 (45.1%) were Colombian mestizos. Twenty-four patients (47.1%) were asymptomatic, and Twenty-seven patients (52.9%) were symptomatic (systemic complications); splenic complications were the most important (85.2%), p=0.0005, and there was a wide spectrum of splenic complications. We found significant elevations in leukocytes, bilirubin, LDH, and CRP. Eighteen patients (66.7%) received medical management, five patients (18.5%) required splenectomy, and only 5.9% of patients were sent for genetic counseling. Conclusions: Splenic complications were the most relevant in symptomatic patients. Most patients received medical treatment, and 18.5% of patients required splenectomy. Our results reflect the absence of redirection of these patients to genetic counseling.

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A Case of Autosplenectomy in Sickle Cell Trait Following an Exposure to High Altitude

Cited by 5 publications

References 28 publications

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

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

Sickle cell trait: a cause of abdominal pain and pulmonary embolism

Sickle Cell Trait, Clinical Manifestations and Outcomes: A Cross-Sectional Study

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