Long-term follow-up of partial splenectomy in Gaucher's disease.
Seven children with Gaucher's disease who underwent partial splenectomy were followed for 7 to 8 years. None of the children had systemic sepsis or symptoms related to liver enlargement. Bone crisis occurred in only two children who had experienced bone crisis prior to partial splenectomy. This contrasts with the development of bone crises in five of six children who underwent total splenectomy at a similar age and who had previously been free of bone symptoms. Partial splenectomy should be regarded as a temporary solution in the treatment of hypersplenism and the mechanical compression related to the huge spleen. In 71% of the patients, massive enlargement and a severe to moderate degree of pancytopenia occurred again after 3 to 8 years; total splenectomy was required in three patients.
Cohen
I J
IJ
Sambur Center for Pediatric Hematology and Oncology, Beilinson Medical Center, Petah Tiqva, Israel.
Katz
K
K
Freud
E
E
Zer
M
M
Zaizov
R
R
D000293:Adolescent; D001842:Bone and Bones; D002648:Child; D005260:Female; D005500:Follow-Up Studies; D005776:Gaucher Disease / Q000473:pathology / Q000503:physiopathology / Q000601:surgery*; D006801:Humans; D008099:Liver / Q000473:pathology; D008297:Male; D010146:Pain / Q000503:physiopathology; D010198:Pancytopenia / Q000628:therapy; D011446:Prospective Studies; D013154:Spleen / Q000473:pathology; D013156:Splenectomy / Q000379:methods*