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Bone marrow transplantation corrects the splenic reticuloendothelial
dysfunction in sickle cell anemia
A Ferster, W Bujan, F Corazza, C Devalck, P Fondu, M Toppet, M Verhas and E Sariban
Pediatric Hematology/Oncology Unit, Hopital Universitaire des Enfants Reine
Fabiola, Brussels, Belgium.
In sickle cell anemia (SCA), the loss of reticuloendothelial function is
the result of vasoocclusive events occurring in the spleen. Such asplenia
occurs early in the course of the disease and is considered to be permanent
in late childhood. In this report, three patients 10, 11, and 14 years of
age suffering from severe SCA and found to be asplenic were treated by bone
marrow transplantation (BMT). Before transplantation, all three patients
had loss of reticuloendothelial splenic function, as assessed by the
presence of abundant Howell-Jolly bodies on blood smears and absence of
technetium 99m (99mTc) splenic uptake. After BMT, Howell-Jolly bodies
disappeared from blood smear, whereas 99mTc isotopic scan found normal
isotope uptake. Our data indicate that BMT can correct "permanent asplenia"
in SCA patients. However, it remains to be determined if such treatment can
also correct other SCA-related organ dysfunctions.
Volume 81,
Issue 4,
pp. 1102-1105,
02/15/1993
Copyright © 1993 by The American Society of Hematology
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