Effects of Chronic Transfusions on Abdominal Sonographic Abnormalities in Children with Sickle Cell Anemia

Abstract

Objective To assess the effects of chronic erythrocyte transfusions on prevalence of sonographic incidence of organ damage in children with sickle cell anemia (SCA). Study design Children (n=148; mean age, 13.0 years) with SCA, receiving chronic transfusions (average, 7 years) underwent abdominal sonography at 25 institutions. After central imaging review, spleen, liver and kidney measurements were compared with published normal values. Potential relations between ultrasound, clinical and laboratory data were explored via Analysis of Variance, Student t-test and Cochran Mantel Haenzel tests of non-zero correlation. Results Average spleen length was similar to normal children, but over one-third had spleen volumes > 300mL, 15 had previous splenectomy for splenomegaly and 24 had abnormal splenic echotexture. Two-thirds had hepatobiliary disease; 37 had prior cholecystectomy, 46 had gallstones, 16 had gallbladder sludge. Gallbladder disease correlated with older age (p = 0.002), longer liver length (p < 0.001), longer duration of transfusions (p = 0.034) and higher total bilirubin (p < 0.001). Liver (p < 0.001) and renal lengths (p ≤ 0.005) were larger than published norms. Conclusions In children with SCA, long-term transfusion therapy may not prevent development or progression of abdominal organ dysfunction.

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

“…In our study, 70% of the patients treated with chronic transfusions had sonographically visible spleens without autosplenectomy, similar to a prior study reporting a rate of almost 80% 15 . We found that patients who were treated with chronic transfusions were more likely to have intrasplenic nodules, and all patients treated with chronic transfusions had echogenic spleens on sonography.…”

“…Hydroxyurea decreases the frequency of functional asplenia among patients with SCD and has been associated with splenomegaly without sequestration 13 , 14 . Long‐term transfusion therapy has not been effectively shown to prevent splenic dysfunction, but in a study of 148 patients age 5 to 19 years with HbSS treated with chronic transfusions, only 20% had surgical splenectomy or autosplenectomy, and more than 33% had splenomegaly 15 . Transfusion therapy can lead to iron overload, which causes the spleen to be hypointense on T2‐weighted magnetic resonance imaging (MRI) 16 .…”

Elective cholecystectomy reduces morbidity of cholelithiasis in pediatric sickle cell disease

“…With such a high percentage of emergent patients receiving transfusions or hydroxyurea, it warrants further prospective study to determine if these patients are at higher risk of hepatobiliary complications irrespective of therapy or if some therapies, as seen in SWiTCH, may accelerate hepatobiliary complications. The higher number of patients on chronic transfusion confirm SWiTCH data that identified that two thirds of patients on chronic transfusion for a mean of 7 years had hepatobiliary disease …”

A study of hepatobiliary involvement in adult patients with sickle cell disease

“…18,19 In our study gall stones were detected in (14%), which is lower than some studies like Gumiero et al in which gall stones were detected in 45% but similar to studies like Mc Carville et al and Bokade in which gall stones were found in 21% and 29% respectively. 20,21 In our study, there were 10 % of patients who had gall bladder sludge, which is similar to studies by Mahebba (12 %) and Dalia (14 %).…”