Is Sickle Cell Trait Really Innocent?

Yeral, Mahmut; Boğa, Can

doi:10.4274/tjh.galenos.2020.2020.0344

Cited by 3 publications

(4 citation statements)

References 6 publications

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“…In paraclinical data, increased bilirubin levels, LDH, reticulocyte count, and sometimes anemia have been described. 18,22,23 By contrast, we found no difference in hemoglobin levels or platelets among the symptomatic or asymptomatic groups; All complications are associated with different pathophysiological conditions in which the leukocyte count can increase, 14 which explains why a clear difference was found in WBC counts, with higher counts in symptomatic patients. However, although hemoglobin levels were similar, an interesting finding was statistically significant higher levels of bilirubin and LDH, possibly reflecting a low grade of hemolysis among "sickling" red blood cells, as described in these patients, which we can associate with the clinical presentation.…”

Section: Methodscontrasting

confidence: 57%

“…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). 24,25 The presence of SCT is established by finding both hemoglobin A (HbA) and hemoglobin S (HbS), with an amount of HbA greater than that of HbS (i.e., HbAS); typically, hemoglobin S levels are between 20% and 45%.…”

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confidence: 99%

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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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Section: Methodscontrasting

confidence: 57%

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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“…Sickle cell trait occurs in around 300 million individuals worldwide [1]. The sickle cell trait affects approximately 13.6 percent of the population in Turkey's Mediterranean region [2]. In contrast to thalassemia carriers, sickle cell carriers can develop clinical symptoms similar to sickle cell anemia (SCA) patients; therefore, it is crucial to identify sickle cell trait individuals [3].…”

Section: Introductionmentioning

confidence: 99%

Diagnosis of a sickle cell carrier while investigating the cause of neutropenia in a child with short stature receiving growth hormone

Kaya

2024

JCLR

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Short stature requiring growth hormone treatment is a well-known complication of sickle cell anemia (SCA). Sickle cell carriers can experience a variety of complications similar to SCA; however, short stature has not been documented in sickle cell carriers yet. Diagnosing using a complete blood count in sickle cell carriers is also difficult. Thus, hemoglobin (Hb) electrophoresis is useful in diagnosing these carriers. We present a case in which a sickle cell carrier is identified using Hb electrophoresis while investigating the etiology of neutropenia in a child using growth hormone for short stature.

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“…8 Individuals with SCT generally do not display the haematological and clinical symptoms of SCA. Indeed, some of them are unaware that they carry the faulty gene; however, there is evidence that they may exhibit complications during stressful situations or life events [9][10][11][12] or vigorous physical activities. 13 Pregnancy is a stressful physiological event, often associated with emotional changes, anxiety, and depression.…”

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confidence: 99%

Adverse Pregnancy Outcomes in Sickle Cell Trait: A Prospective Cohort Study Evaluating Clinical and Haematological Parameters in Postpartum Mothers and New-Borns.

Al-Kindi¹

2022

Mediterr J Hematol Infect Dis

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– Sickle cell trait (SCT) is a condition caused by the inheritance of a single allele of the abnormal haemoglobin beta gene, HbS. Carriers of SCT are generally asymptomatic, and they do not manifest the haematological and clinical abnormalities of sickle cell anaemia (SCA). However, there is evidence that they display some of the symptoms in stressful situations. Pregnancy is a stressful physiological event, and it is not clear if SCT adversely affects pregnancy outcomes, particularly those from the developing countries, who regularly suffer from nutritional insufficiency. Objective – This study aims to investigate pregnancy outcomes in Sudanese women with SCT. Subjects and methods – Pregnant women with (HbAS, n=34) and without (HbAA, n=60) SCT were recruited during their first trimester by El Obeid Hospital, Kordofan, Western Sudan. Ethical approval from the Faculty of Medicine, University of Khartoum, and informed consent from the participants were obtained. Detailed anthropometric, haematological, clinical, obstetric and birth outcome data were documented. Blood samples were collected at enrolment and delivery. Results – At enrolment, the first trimester, the SCT group did not manifest SCA symptoms, and there was no difference in any of the haematological parameters between the SCT and control groups. At delivery, the women with SCT compared with the control group had lower levels of haemoglobin (Hb, p=0.000), packed cell volume (PCV, p=0.000), mean corpuscular haemoglobin (MCH, p=0.002) and neutrophil counts (p=0.045) and higher mean corpuscular volume (MCV, p=0.000) and platelet counts (p=0.000). Similarly, at delivery, the babies of SCT women had lower birth weight (p=0.000), lower Hb (p=0.045), PCV (p=0.000), MCH (p=0.000) and higher neutrophil (p=0.004) and platelet counts (p=0.000) than the babies of the healthy control group. Additionally, there were more miscarriages, stillbirths and admissions to the Special Care Baby Unit (SCBU) in the SCT group. Conclusions – The study revealed that SCT is associated with adverse pregnancy outcomes, including maternal and neonatal anaemia, low birth weight and increased risk of still birth, miscarriage and admission to SCBU. Therefore, pregnant women with SCT should be regarded as a high-risk group, and given pre-conceptual advice and multidisciplinary antenatal and postnatal care.

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Is Sickle Cell Trait Really Innocent?

Cited by 3 publications

References 6 publications

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

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

Diagnosis of a sickle cell carrier while investigating the cause of neutropenia in a child with short stature receiving growth hormone

Adverse Pregnancy Outcomes in Sickle Cell Trait: A Prospective Cohort Study Evaluating Clinical and Haematological Parameters in Postpartum Mothers and New-Borns.

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