Expected Sonographic Appearance of the Spleen in Children and Young Adults With Sickle Cell Disease

Abstract

Twenty years ago, children with HbSS SCD were expected to have autosplenectomy by age 5 years. There have been changes in the radiologic appearance of the spleen in patients with SDC, likely due to improved supportive care and the use of acute and chronic transfusion therapy. We found that autosplenectomy is rare by age 5 years, and during childhood and adolescence, the spleen typically appears echogenic, heterogeneous, or both, depending on disease severity.

Serum Immunoglobulin Levels in Children with Sickle Cell Disease: A Large Prospective Study

“…In these studies, the authors suggested a correlation between loss of splenic tissue and low IgM concentration [10]. Surprisingly, although loss of splenic function occurs later in life and to a lesser extent in HbSC than HbSS [5,6], we found no significant difference in IgM level between the two sickle subtype groups. Moreover, therapeutic intensification (by TP or HU) did not prevent decreased IgM level.…”

“…Another study, investigating the mechanical filtration ability of the spleen by using 99 m Tc heated RBC scintigraphy in a cohort of 57 infants with sickle cell anemia, showed that in 32% and 50% of infants, splenic function was decreased at 6 and 18 months of age, respectively [3]. The age varies for irreversible fibrosis of the spleen (a loss also referred to as autosplenectomy), and changes have been described over time, likely due to improved supportive care and the use of acute and chronic transfusion therapy [5]. On ultrasonography, autosplenectomy was found in 39.5% of a cohort of 86 patients with HbSS who were 0–21 years of age but in only 5% of children less than 5 years of age [5].…”

“…The age varies for irreversible fibrosis of the spleen (a loss also referred to as autosplenectomy), and changes have been described over time, likely due to improved supportive care and the use of acute and chronic transfusion therapy [5]. On ultrasonography, autosplenectomy was found in 39.5% of a cohort of 86 patients with HbSS who were 0–21 years of age but in only 5% of children less than 5 years of age [5].…”

“…None of the 59 children less than 4 years of age had abnormally high pitted cells, whereas the count increased in 19 of 96 (22%) children with 4–12 years of age and 25 of 56 (46%) older than 12 years of age [6]. In HbSC patients, autosplenectomy was found in only 3 of 21 patients (14.3%) with 0–21 of age [5]. Splenic loss of function occurs with milder SCD genotypes, however later in life, and to a lesser extent than in patients with severe SCD genotypes.…”

“…This function is essential to prevent inflammatory pathology and the development of autoimmunity. Of note, antinuclear antibody positivity is more common in SCD, and a higher than expected incidence of connective tissue diseases has been reported in patients with HbSS subtypes as compared with the general population [2,3,4,5,6,7,8,9].…”