Banti's syndrome

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Mahshid Mir, M.D. [2]

Historical Perspective

• Banti's syndrome was first described in 1883 by Dr. Banti, an Italian physycian. He described a case series of patients probably exposed to an unknown toxin leading to chronic spleen and liver injury. Patients were developing chronic splenomegaly followed by secondary hypochromic anemia. The disease progressed to advanced stages and manifested by hemorrhages from gastrointestinal tract, ascites, and advanced liver injury and cirrhosis.
• The most important pathologic finding in Banti's syndrome is called "fibro-ad\l=e'\nie"in the splenic white pulp or splenic lymphoid nodules
  • This process involves having its inception around the splenic penicillate arteries.
• Osler support and further defined Banti's concept of the disease. He defined the diseaseas: "An intoxication of unknown nature characterized by great chronicity. There is a preliminary progressive enlargement of the spleen which cannot be correlated with any known cause, anemia of secondary type with leukopenia, a marked tendency to hemorrhage from the lower esophagus, and a terminal state with cirrhosis of the liver."

• The disease got further attention and other physicians explained an association between splenomegaly and secondary anemia with splenic or the portal vein thrombosis.
• Cauchois, Warthin, Eppinger, and Klemperer further explored the disease and showed that the thrombosis is the main cause of the splenomegaly.
• Larrabee evaluated forty seven cases of Banti's syndrome and concluded the main clinical symptoms of Banti's syndrome is dependent on intra-abdominal lesion obstructing the venous outflow of the spleen.He notices that liver cirrhosis is the most common cause of splenic venous outflow obstruction. He suggested the use of term "chronic congestive splenomegaly" as an alternative name for "Banti's syndrome".
• Rousselot was the one who brought attention to the gross pathology of portal veins when a splenectomy was being performed on patients with Banti's disease. The gross pathology of portal veins showing dilated and tortuous veins in the splenic pedicle, leading to dilation of slenic collateral circulation. As there was enormous adhesion around the spleen, splenectomy was a dangerous procedure for these patients.
• Thompson explained similarities in the microscopic pathology of spleen in patients with Banti's syndrome, characterized by:
  • Variable degrees of follicular atrophy
  • Fibrosis of the white pulp
  • Dilated venous sinuses
  • Perifollicular hemorrhages
• He described the obstructive lesions as being either intrahepatic or extrahepatic.
  • The intrahepatic lesion was defined to be cirrhosis and contained 68% of the cases that has undergone splenectomy.
• Thompson and his associates shave made observations on the splenic vein pressure in fifteen cases in the course of operation by inserting the needle of the venous pressure apparatus into the splenic vein. The relatively great increase in splenic pressure in cases of Banti's syndrome when compared with the venous pressure simultaneously recorded in the arm suggests that portal hypertension is an important factor in the production of the chronic splenomegaly. The contention of the workers of the Combined Spleen Clinic is that Banti's disease or splenic anemia is a secondary mechanical manifestation of any of a variety of lesions producing chronic splenic vein hypertension. They therefore suggest that the terms Banti's disease and splenic anemia be replaced by congestive splenomegaly, as originally suggested by Larrabee. The type of liver cirrhosis in prolonged schistosomiasis results in the greatest increase in portal pressure and the largest spleens. The behavior of this parasitic disease is ideal for the production of portal hypertension.
• Campbell was working on cases with schistosomiasis in the Orient. He described schistosomiasis as the main cause of the manifestations of Banti's syndrome. He believed that as most of the cases were observed by Banti in Italy, and based on the geographical proximity of Italy to Egypt which is considered as high prevalent area for schistosomiasis , probably the schistasomiasis is the main reason of the disease.