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Spleen function in children with sickle B+ thalassemia
N J Barrios1, D V Kirkpatrick, D Lohman
1Department of Pediatric Hematology/Oncology, Tulane University Medical School, New Orleans, LA 70112.
Journal of the National Medical Association
|September 1, 1991
Summary
Children with sickle B+ (SB+) thalassemia show normal splenic function and a low risk of infection. This suggests avoiding routine prophylactic penicillin in young SB+ thalassemia patients.
Area of Science:
- Hematology
- Pediatrics
- Nuclear Medicine
Background:
- Splenic dysfunction is common in hemoglobinopathies but poorly understood in sickle B+ (SB+) thalassemia.
- Assessing splenic function is crucial for managing infection risk in these patients.
Purpose of the Study:
- To evaluate the clinical course and splenic function in children with SB+ thalassemia.
- To determine the risk of infection and vaso-occlusive episodes in this cohort.
Main Methods:
- Technetium sulfur colloid spleen scans were performed simultaneously with pitted erythrocyte analysis using direct interference contrast microscopy (DICM).
- Evaluated 12 children with SB+ thalassemia and compared results to normal controls and sickle cell patients.
Main Results:
- All SB+ thalassemia patients exhibited normal radiocolloid uptake, indicating preserved splenic function.
- The mean percentage of pitted erythrocytes (2.2%) was slightly elevated compared to controls (0.06%) but significantly lower than sickle cell patients (13.8%).
- No bacteremia/sepsis episodes and a low prevalence of vaso-occlusive episodes were observed over 136 patient-years.
Conclusions:
- SB+ thalassemia in children does not appear to cause clinically significant splenic dysfunction.
- Children with SB+ thalassemia younger than 10 years have a low risk of invasive bacterial infections, similar to eusplenic children.
- Conservative management of febrile episodes and avoidance of prophylactic penicillin are recommended for these patients.