Treatment of the thalassemia syndrome with splenectomy.
Splenectomy was performed on 152 patients with thalassemia (thal), including 90 cases with Hb H disease, 48 cases with homozygous beta-thal, and 14 cases with Hb E-beta-thal. The therapeutic effect in Hb H disease was 83.3%, and 32.3% in Hb E-beta-thal and beta-thal. In Hb H disease, the hemoglobin (Hb) level increased 30 g/l in 14 of 29 cases. After splenectomy, the life-span (T1/2) of 51Cr RBC in Hb H and Hb E-beta-thal increased to 18.2 +/- 2.6 and 18.2 days, respectively. The number of inclusion (Heinz) bodies attached to the RBC membrane increased after surgery. The interdermal delayed supersensitivity reaction became negative in seven of 26 cases of Hb H disease; the IgG also decreased after surgery. The number of PAS positive foam cells in the spleen correlated to Hb level after surgery. Infection is a common complication.
Yang
X Y
XY
Institute of Hematology, Chinese Academy of Medical Sciences, Tianjin, P.R. China.
Qu
Q
Q
Yang
T Y
TY
Chan
W C
WC
Chu
J X
JX
Chen
Z
Z
Ning
A L
AL
Liu
F J
FJ
Lin
Z X
ZX
Zhou
Y L
YL
D002681:China; D004905:Erythrocyte Aging; D006801:Humans; D008854:Microscopy, Electron; D011183:Postoperative Complications; D013156:Splenectomy; D013789:Thalassemia / Q000601:surgery*