Pediatric Sickle Cell Disease Patients on Hydroxyurea Have Higher Rates of Surgical Splenectomy

Menchaca, Alicia D; Style, Candace C.; Villella, Anthony; Burdjalov, Maria; Minneci, Peter C.

doi:10.1016/j.jss.2022.11.026

Cited by 5 publications

(3 citation statements)

References 13 publications

Supporting

Mentioning

Contrasting

Order By: Relevance

“…In a retrospective study of 28,580 patients in the Pediatrics Health Information System database, the prevalence of splenectomy was 7.2% among patients on hydroxyurea therapy compared to 3.2% in those not on therapy. The age of splenectomy was also lower in the treated group 18 . In a cohort of 23 patients with Hb SS and Hb Sβ 0 treated with hydroxyurea, the onset of ASSC was noted at substantially older ages than has been previously reported; the authors hypothesize that preserved splenic function on hydroxyurea therapy may be responsible for this delayed onset of ASSC 19 .…”

Section: Discussionmentioning

confidence: 77%

Splenic Complications in Pediatric Sickle Cell Disease: A Retrospective Cohort Review

George,

Conneely,

Mangum

et al. 2024

Preprint

View full text Add to dashboard Cite

Objective: To delineate the natural history of splenic complications other than the loss of splenic function in children with sickle cell disease (SCD), we performed a retrospective chart review of patients with SCD treated at the Texas Children’s Hospital. Methods: We determined the dates of diagnoses of splenic complications, the number of ASSC events, and hydroxyurea treatment in patients with SCD. We also examined the association of hydroxyurea therapy with the onset and severity of ASSC. Results: The cumulative prevalence of splenic complications was 24.7% for splenomegaly, 24.2% for ASSC, 9.6% for hypersplenism, and 5.6% for splenectomy. The cumulative prevalence of all splenic complications was highest in patients with hemoglobin Sβ (69.2%), intermediate in hemoglobin SS (33.3%), low in hemoglobin SC (9.0%), and non-existent in hemoglobin Sβ . The overall event-rate of ASSC was 8.3 per hundred patient-years. The event-rate was 28.4 in the hemoglobin Sβ , 10.9 in hemoglobin SS, and 3.5 in hemoglobin SC Patients with hemoglobin SS and hemoglobin Sβ on hydroxyurea therapy had a significantly higher occurrence of ASSC than those who were not, with event-rates of 14.2 and 3.1, respectively. The event-rate was also higher for children who started hydroxyurea before age 2 years than for those who started after this age (19.8 and 9.2 respectively). Conclusions: The prevalence and severity of splenic problems vary widely between different sickle cell genotypes, with hemoglobin Sβ having the most severe complications. Hydroxyurea therapy is strongly associated with incidence of ASSC, particularly when initiated before two years of age.

show abstract

Section: Discussionmentioning

confidence: 77%

Splenic Complications in Pediatric Sickle Cell Disease: A Retrospective Cohort Review

George,

Conneely,

Mangum

et al. 2024

Preprint

View full text Add to dashboard Cite

show abstract

“…Hydroxyurea has undergone extensive experimental tests including infants, children, and adults, with sickle cell anemia (SCA). These trials consistently show that hydroxyurea is effective in reducing pain, episodes of pulmonary crisis, the requirement for blood infusions, and hospitalizations in individuals with SCA [23]. Hydroxyurea therapy is safe to start at any age, including infants as young as 6 -9 months old.…”

Section: The Treatment Indications For Hydroxyureamentioning

confidence: 89%

“…Increasingly, families are requesting hydroxyurea treatment, particularly when one sibling is already receiving it. In these cases, practitioners are required to carefully assess potential advantages for patients who have not yet shown signs of severe symptoms[23].…”

mentioning

confidence: 99%

Review on Hydroxyurea Usage in Young Children with Sickle Cell Disease: Examining Hemoglobin Induction, Potential Benefits, Responses, Safety, and Effectiveness

Mkwambe,

Deng,

Zhao

2024

IJCM

View full text Add to dashboard Cite

Sickle cell disease (SCD) is a prevalent condition, particularly in the countries of sub-Saharan Africa, where the presence of specific genes associated with Malaria contributes to its high prevalence. Patients with sickle cell disease frequently experience painful episodes necessitating hospitalization, and their hemoglobin levels are typically lower than those of the general population. There are different treatment options available to manage complications, such as transfusing blood, hydroxyurea, and strong anti-pains. However, with all these treatments, patients still commonly experience pain crises and suffer from organ damage. Hydroxyurea, the sole approved medication for sickle cell anemia in developed and developing countries, is widely used in children despite being primarily indicated for adults. Multiple studies have demonstrated the efficacy of hydroxyurea in inducing HbF production in young children with SCD. Elevated HbF levels have been associated with improved clinical outcomes, including a reduction in vaso-occlusive crises, acute chest syndrome, and the need for blood transfusions. Furthermore, increased HbF levels have been shown to ameliorate disease-related organ damage, such as pulmonary hypertension and sickle cell retinopathy. The response to hydroxyurea treatment in young children with SCD is variable. Some patients achieve substantial increases in HbF levels and experience significant clinical benefits, while others may have a more modest response. Factors influencing the response include baseline HbF levels, genetic modifiers, treatment adherence, and dose optimization. Safety is a crucial consideration when using hydroxyurea in young children. Studies have shown that hydroxyurea is generally well-tolerated, with the most common adverse effects being myelosuppression, gastrointestinal symptoms, and dermatological manifestations. However,

show abstract

Splenic complications in pediatric sickle cell disease: A retrospective cohort review

George,

Conneely,

Mangum

et al. 2024

Pediatric Blood & Cancer

View full text Add to dashboard Cite

ObjectiveTo delineate the natural history of splenic complications other than loss of splenic function in children with sickle cell disease (SCD), we performed a retrospective chart review of patients with SCD treated at the Texas Children's Hospital.MethodsWe determined the dates of diagnoses of splenic complications, the number of acute splenic sequestration crises (ASSC), and hydroxyurea treatment in pediatric patients with SCD. We also examined the association of hydroxyurea therapy with the onset and severity of ASSC.ResultsThe cumulative prevalence of splenic complications was 24.7% for splenomegaly, 24.2% for ASSC, 9.6% for hypersplenism, and 5.6% for splenectomy. The cumulative prevalence of splenic complications was highest in patients with hemoglobin Sβ0 (69.2%), intermediate in hemoglobin SS (33.3%), low in hemoglobin SC (9.0%), and non‐existent in hemoglobin Sβ+. The overall event rate of ASSC was 8.3 per 100 patient‐years. The event‐rate was 28.4 for hemoglobin Sβ0, 10.9 for hemoglobin SS, and 3.5 for hemoglobin SC. Patients with hemoglobin SS and hemoglobin Sβ0 on hydroxyurea therapy had a significantly higher occurrence of ASSC than those who were not, with event rates of 14.2 and 3.1, respectively. The event rate was also higher for children who started hydroxyurea before age 2 years than for those who started after this age (19.8 and 9.2, respectively).ConclusionsThe prevalence and severity of splenic problems vary widely between different sickle cell genotypes, with hemoglobin Sβ0 having the most severe complications. Hydroxyurea therapy is associated with increased incidence of ASSC, particularly when initiated before 2 years of age.

show abstract

Pediatric Sickle Cell Disease Patients on Hydroxyurea Have Higher Rates of Surgical Splenectomy

Cited by 5 publications

References 13 publications

Splenic Complications in Pediatric Sickle Cell Disease: A Retrospective Cohort Review

Splenic Complications in Pediatric Sickle Cell Disease: A Retrospective Cohort Review

Review on Hydroxyurea Usage in Young Children with Sickle Cell Disease: Examining Hemoglobin Induction, Potential Benefits, Responses, Safety, and Effectiveness

Splenic complications in pediatric sickle cell disease: A retrospective cohort review

Contact Info

Product

Resources

About