GAUCHER'S DISEASE: I. A CASE WITH HEMOLYTIC ANEMIA AND MARKED THROMBOPENIA; IMPROVEMENT AFTER REMOVAL OF SPLEEN WEIGHING 6822 GRAMS: II. LIPID ANALYSIS OF THE GAUCHER'S SPLEEN

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Abstract
A case of Gaucher''s disease in a cf of 41 is reported. The diagnostic problem was one of hemolytic anemia complicated by a huge splenomegaly. In favor of hemolytic jaundice were: a tower skull (oxycephalus); anemia; leukocytosis; elevated icterus index; and some spherocytosis. On the other hand, the huge splenomegaly; the universal pigmentation of the skin; the symmetrical pigmentation of the legs; and the prominent pingueculae pointed to Gaucher''s disease. A thrombocytopenia was considered confirmatory. Radiographic studies of the bones were not helpful. Repeated myelograms failed to reveal even a single Gaucher cell. The progressive hemolytic anemia was the indication for splenectomy, the spleen on removal weighing 6,822 gs. Following this the Hb and erythrocyte values rose steadily, reaching and maintaining normal values within 5 mos. The pigmentation was no longer evident except for some areas on the legs; the pingueculae disappeared. Radiographic studies of the bones 19 mos. after splenectomy showed no disease. The characteristic lipoid constituent, kerasin, was isolated from the spleen. An increase of the cerebrosides at the expense of neutral fat was observed.

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