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Rapidly Progressive Type III Gaucher Disease: Deterioration Following Partial Splenectomy
Author(s) -
KYLLERMAN M.,
CONRADI N.,
MÅNSSON J. E.,
PERCY A. K.,
SVENNERHOLM L.
Publication year - 1990
Publication title -
acta pædiatrica
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.772
H-Index - 115
eISSN - 1651-2227
pISSN - 0803-5253
DOI - 10.1111/j.1651-2227.1990.tb11492.x
Subject(s) - splenectomy , medicine , gaucher's disease , spleen , disease , bone marrow , gastroenterology , surgery , pathology
. Total splenectomy has been found to accelerate disease progression in Type III Gaucher disease, therefore partial splenic resection was performed in a 28 month old girl with rapidly progressive (non‐Norrbottnian) Type III disease to alleviate the effects of hypersplenism. Surgery failed to arrest the disease process and splenomegaly recurred within three months. The erythrocyte glucosylceramide level increased post‐operatively. Post‐mortem studies revealed dense accumulation of Gaucher cells in spleen, liver, lungs, bone marrow and within the central nervous system. The glucosylceramide content of brain and liver was elevated to the range previously noted in splenectomised Norrbottnian patients. Thus, in rapidly progressive Type III Gaucher disease, neither total nor partial splenectomy alone provides a favourable outcome.