Spleen Function in Children With S-Beta +Thalassemia (Sb+ Thal)

Barrios, Nilka J.; Humbert, Jean-Paul; Kirkpatrick, Dahlia; Wilson, William S.; Jones, Malita A.; Stern, Martin

doi:10.1097/00043426-199021000-00039

Cited by 2 publications

(3 citation statements)

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“…Conversely, children with Hb Sβ + had virtually no evidence of splenic disease, even on extended analysis into late adolescence. Our data on Hb SC and Hb Sβ + patients correspond with those from earlier studies, which indicated that Hb SC patients have progressive splenic disease throughout life while patients with Hb Sβ + mostly remain disease-free 4,5 .…”

Section: Discussionsupporting

confidence: 90%

“…This progressive loss of splenic function is associated with impaired ability to clear encapsulated bacteria from the blood and an increased risk of bacteremia. Loss of splenic function occurs later in life in people with hemoglobin SC disease (Hb SC) and may not occur at all in those with sickle beta-plus thalassemia disease (Hb Sβ + ) 4,5 .…”

Section: Introductionmentioning

confidence: 99%

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

George,

Conneely,

Mangum

et al. 2024

Preprint

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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.

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Section: Discussionsupporting

confidence: 90%

Section: Introductionmentioning

confidence: 99%

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

George,

Conneely,

Mangum

et al. 2024

Preprint

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“… 19 – 22 Patients with Hb SB+ thalassemia may also suffer from dysfunctional splenic function but this research is limited. 23 This retrospective cohort did not demonstrate an association between splenomegaly noted on clinical exam with thrombocytopenia and HU dose. However, future studies of HU in this population should include a more rigorous method for monitoring splenic function and size.…”

Section: Discussionmentioning

confidence: 52%

Hydroxyurea decreases hospitalizations in pediatric patients with Hb SC and Hb SB+ thalassemia

Lebensburger¹,

Patel²,

Palabindela³

et al. 2015

JBM

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PurposePatients with hemoglobin SC (Hb SC) and hemoglobin SB+ (Hb SB+) thalassemia suffer from frequent hospitalizations yet strong evidence of a clinical benefit of hydroxyurea (HU) in this population is lacking. Patients with recurrent hospitalizations for pain crisis are offered HU at our institution based on small cohort data and anecdotal benefit. This study identifies outcomes from a large cohort of patients with Hb SC and SB+ thalassemia who were treated with HU for 2 years.Materials and methodsA retrospective review was conducted of 32 patients with Hb SC and SB+ thalassemia who were treated with HU. We reviewed the number, and reasons for hospitalization in the 2 years prior to, and 2 years post-HU treatment as well as laboratory changes from baseline, over 1 year.ResultsPatients with Hb SC and SB+ thalassemia started on HU for frequent pain, had a significant reduction in hospitalizations over 2 years as compared to the 2 years prior to HU initiation (mean total hospitalizations/year: pre-HU: 1.6 vs post-HU 0.4 hospitalizations, P<0.001; mean pain hospitalizations/year: pre-HU 1.5 vs post-HU 0.3 hospitalizations, P<0.001). Patients demonstrated hematologic changes including an increase in percent fetal hemoglobin (%HbF) pre–post HU (4.5% to 7.7%, P=0.002), mean corpuscular volume (74 to 86 fL, P<0,0001), and decrease in absolute neutrophil count (5.0 to 3.2×109/L, P=0.007). Patients with higher doses of HU demonstrated the greatest reduction in hospitalizations but this was unrelated to absolute neutrophil count.ConclusionThis cohort of patients with Hb SC and SB+ thalassemia provides additional support for using HU in patients with recurrent hospitalizations for pain. A large randomized multicenter trial of HU to reduce pain admissions should be conducted to confirm these data and provide much needed evidence based recommendations for this population.

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Spleen Function in Children With S-Beta +Thalassemia (Sb+ Thal)

Cited by 2 publications

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

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

Hydroxyurea decreases hospitalizations in pediatric patients with Hb SC and Hb SB+ thalassemia

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