Bone marrow transplantation corrects the splenic reticuloendothelial dysfunction in sickle cell anemia.
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.
Ferster
A
A
Pediatric Hematology/Oncology Unit, Hôpital Universitaire des Enfants Reine Fabiola, Brussels, Belgium.
Bujan
W
W
Corazza
F
F
Devalck
C
C
Fondu
P
P
Toppet
M
M
Verhas
M
M
Sariban
E
E
D000293:Adolescent; D000755:Anemia, Sickle Cell / Q000097:blood / Q000503:physiopathology / Q000601:surgery*; D016026:Bone Marrow Transplantation; D002648:Child; D002675:Child, Preschool; D004908:Erythrocyte Inclusions / Q000473:pathology; D006801:Humans; D012157:Mononuclear Phagocyte System / Q000503:physiopathology*; D011877:Radionuclide Imaging; D013154:Spleen / Q000000981:diagnostic imaging / Q000503:physiopathology*; D013667:Technetium
D013667:Technetium