GAUCHER'S DISEASE AND PREGNANCY: TWO CASE REPORTS, INCLUDING OBSERVATIONS ON THE EFFECTS OF ADRENAL STEROIDS

Abstract

A therapeutic abortion followed by splenectomy was performed on a young woman with Gaucher''s disease who had had 1 previous normal pregnancy. She had 2 subsequent normal pregnancies. Five years later, she and her 3 children are in good health. Gaucher''s disease was demonstrated in a 2d case following a normal delivery. Cortisone induced partial remission of her "hypersplenic" panhemocytopenia, while subsequent splenectomy induced complete remission. There was postoperative shock requiring reexploration and transfusions. Three months later febrile hepatocellular jaundice appeared and apparently induced an early spontaneous abortion. Survey of the reported cases of Gaucher''s disease with pregnancy revealed an early spontaneous abortion, a premature but otherwise normal delivery complicated by a post partum hemolytic crisis and a series of 7 women who had 9 normal pregnancies and 4 therapeutic abortions. Interruption is not thought justified in Gaucher''s disease.