SPLENOMEGALY OF GAUCHER, AND CHRONIC ACHOLURIC ICTERUS WITH OR WITHOUT ENLARGED SPLEEN In addition to morbus Banti there have been quite recently two new diseases of the blood described, which are perhaps identical, as they certainly strongly resemble each other as well as morbus Banti.
I. Splenomegaly is characterized by an enlarged spleen, lasting for years (up to 39 years!), often congenital, associated with enlarged liver, but never with ascites, and often with a brown pigmenta tion of the skin. The disease is frequently found to be hereditary. The examination of the blood shows nucleated erythrocytes, also megalo blasts, the color index is increased and the blood picture of the white corpuscles was found to vary. Anatomically groups of endothelial-like cells are found in the spleen, liver, lymph-glands and in the bone marrow. It is, therefore, a systemic disease (Schlagenhaufer). Not infrequently tuberculosis is associated with the disease.
II. Another disease, known as chronic acholuric icterus, is char acterized by chronic icterus, urobilinuria and enlargement of the spleen. The latter symptom is not always present. Frequently the liver is enlarged and changes in the blood take place in certain cases, such as a diminution of the red corpuscles, presence of nucleated red cells, also megaloblasts, slight leuktemia with neutrophilic cells predominating, also myeloeytes. The blood serum is icteric, the urine free from biliary coloring matter, but it does contain urobilin and urobilinogen. The stools are choleric. The affection is chronic, causing but slight consti tutional disturbances, and there appears to be a family predisposition to the disease.
French authors associate the symptom-complex with a disease of the liver, but German authors on the other hand, among them Benjamin and Sluka, believe it to be an affection of the htematopoietic system. In their opinion there is an association of the systemic disease with an increased destruction of blood elements, characterized by marked siderosis in the organs and perhaps by increased excretion of uric acid. They regard icterus as the consequence of the destruction of eryth rocytes.
We would again refer to the statements of Bleichroeder mentioned in connection with morbus Banti. In cirrhosis of the liver similar changes have been observed in the gastric mucous membrane as in diseases of the blood, such as a marked deposit of ferruginous pigments and many neutrophilic cells and hyaline bodies, also a soft spleen, frequently with large cells resembling Tiirk's irritative forms, and iron-containing pig ment, the red bone marrow in the thigh with Charcot-Leyden crystals. Cirrhosis of the liver also causes increased excretion of uric acid in its early stage and the icterus is also an urobilin-icterus (cythtemolitic), frequently there exists anremia. Thus we must admit a striking similar ity of the disease with cirrhosis of the liver. Bleichrocder has never found convincing evidence of a primary disease of the hepatic cells in cirrhosis of the liver and is, therefore, inclined to include liver cirrhosis among the diseases of the blood. Perhaps there exists a uniform cause for the occurrence of the changes in the liver and the htematopoietic systern.