TY - JOUR

T1 - The significance of spleen size in children with sickle cell anemia

AU - Nardo-Marino, Amina

AU - Glenthøj, Andreas

AU - Brewin, John N.

AU - Petersen, Jesper

AU - Braunstein, Thomas H.

AU - Kurtzhals, Jørgen A.L.

AU - Williams, Thomas N.

AU - Rees, David C.

N1 - Funding Information: The authors would like to thank associate Professor Christian Pállson Nolsøe (Centre for Surgical Ultrasound, Department of Surgery, Zealand University Hospital, Køge, Denmark) and Professor Paul Sidhu (Department of Radiology, King's College Hospital, London, United Kingdom) for their guidance and supervision in performing abdominal ultrasound examinations. This study was funded by the Danish Centre for Haemoglobinopathies, Copenhagen University Hospital, Rigshospitalet. Thomas N. Williams is supported by a Senior Research Fellowship from the Wellcome Trust (Grant number 202800/Z/16/Z). This research was funded in whole, or in part, by the Wellcome Trust (202800/Z/16/Z and 091758/Z/10/Z). Publisher Copyright: © 2022 The Authors. American Journal of Hematology published by Wiley Periodicals LLC.

PY - 2022

Y1 - 2022

N2 - It is well established that splenic dysfunction occurs in early childhood in sickle cell anemia (SCA), although the determinants and consequences of splenic injury are not fully understood. In this study, we examined spleen size and splenic function in 100 children with SCA aged 0–16 years at King's College Hospital in London. Spleen size was assessed by abdominal ultrasound (US) and splenic function by pitted red blood cells (PIT counts). In our cohort, 5.6% of children aged 6–10 years and 19.4% of children aged 11–16 years had no visible spleen on US (autosplenectomy). Splenomegaly was common in all age groups, with 28% of children overall having larger spleens than the average for their age. Only one child had a PIT count suggesting preserved splenic function. We found no correlation between hemoglobin F levels and spleen size, nor was there any difference in spleen size between children treated with or without hydroxyurea. Although there was a trend toward increased spleen length in children with co-inherited α-thalassemia, this did not reach statistical significance. Finally, we found a strong association between erythrocyte deformability measured with oxygen gradient ektacytometry, spleen size, and PIT counts. In conclusion, our results do not agree with the general perception that most children with SCA undergo autosplenectomy within the first decade of life and indicate that loss of erythrocyte deformability contributes to loss of splenic filtration capacity in SCA, as well as phenotypical variations in spleen size.

AB - It is well established that splenic dysfunction occurs in early childhood in sickle cell anemia (SCA), although the determinants and consequences of splenic injury are not fully understood. In this study, we examined spleen size and splenic function in 100 children with SCA aged 0–16 years at King's College Hospital in London. Spleen size was assessed by abdominal ultrasound (US) and splenic function by pitted red blood cells (PIT counts). In our cohort, 5.6% of children aged 6–10 years and 19.4% of children aged 11–16 years had no visible spleen on US (autosplenectomy). Splenomegaly was common in all age groups, with 28% of children overall having larger spleens than the average for their age. Only one child had a PIT count suggesting preserved splenic function. We found no correlation between hemoglobin F levels and spleen size, nor was there any difference in spleen size between children treated with or without hydroxyurea. Although there was a trend toward increased spleen length in children with co-inherited α-thalassemia, this did not reach statistical significance. Finally, we found a strong association between erythrocyte deformability measured with oxygen gradient ektacytometry, spleen size, and PIT counts. In conclusion, our results do not agree with the general perception that most children with SCA undergo autosplenectomy within the first decade of life and indicate that loss of erythrocyte deformability contributes to loss of splenic filtration capacity in SCA, as well as phenotypical variations in spleen size.

U2 - 10.1002/ajh.26703

DO - 10.1002/ajh.26703

M3 - Journal article

C2 - 36054667

AN - SCOPUS:85139018657

VL - 97

SP - 1520

EP - 1528

JO - American Journal of Hematology

JF - American Journal of Hematology

SN - 0361-8609

IS - 12

ER -