Banti's syndrome
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Template:Your Name
Overview
Historical Perspective
In 1883 Banti described a group of cases characterized by primary enlargement of the spleen, a more or less characteristic secondary hypochromic anemia and a chronic course. The disease passed through typical stages characterized in its advanced stage by hemorrhages from the gastrointestinal tract, ascites and terminal liver cirrhosis. The causative factor was an unknown toxin which acted primarily on the spleen and secondarily on the liver. The essential and distinctive pathologic feature of the spleen was "fibro-ad\l=e'\nie"in the malpighian bodies, the process having its inception around the penicillate arteries. This concept received its support from Osler, who defined the new disease entity as follows : "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 idiopathic nature of Banti's disease soon came into question. Numerous observations appeared demonstrating that splenomegaly and secondary anemia were associated with thrombosis of the splenic or the portal vein. Cauchois 1 collected reports of a number of cases, including his own, of splenomegaly and anemia of pylethrombotic origin. He emphatically refuted Banti's concept. Warthin 2 concluded on the basis of several observations that "every factor of the symptom complex that Osier lays down as the criterion for splenic anemia is also a factor in the complex of portal sclerosis and thrombosis." Eppinger in his classic treatise on hepatolienal diseases devotes a chapter to the relationship between portal congestion and splenomegaly. Klemperer 3 reported a case of cavernomatous transformation of the portal vein presenting clinical and pathologic characteristics corresponding in all respects to that of Banti's. Larrabee,4 on the basis of a study of fortyseven cases, concluded that in the majority of cases presenting the clinical picture of Banti's disease the condition is dependent on some intra-abdominal lesion
obstructing the venous outflow of the spleen. The commonest of these was liver cirrhosis. He states that "we are forced to the conclusion that most if not all of these cases were not only associated with lesions interfering with the outflow of blood from the spleen but were actually the results of such lesions." .Larrabee suggested that the syndrome be referred to as chronic congestive splenomegaly. Johnston,5 in his study on the relative size of the hepatic portal radicles in splenic anemia and Banti's disease, concluded "We are dealing here with mechanical alterations of the portal blood flow interfering with the free exit of blood from the spleen." Interesting contributions have recently come from he Combined Spleen Clinic at the Presbyterian Hospital, New York. Rousselot6 calls attention to the fact that the appearance of the portal circulation in the course of a splenectomy for Banti's disease is at once indicative of portal hypertension. This dilated and tortuous collection of veins in the splenic pedicle and the enormously dilated veins of the large collateral circulation in the adhesions surrounding the spleen make the removal of the spleen in these cases a formidable and hazardous procedure. This local vascular condition, Rousselot points out, was present regardless of the nature of the "obstructive factor." Microscopic examination of the removed spleens revealed the essential similiarity of the changes in the various cases. Thompson of the same clinic sums up the experience of the clinic comprising 137 cases observed in the past ten years. Of this group 100 cases had been followed under nearly ideal circumstances for clinical investigation. Splenectomies were done in sixty-eight cases. The cases were those of splenomegaly with anemia, leukopenia and thrombocytopenia, development of increased collateral circulation between the portal and peripheral venous circulation and microscopic changes in the spleen that are characteristic. The result of this study is a refutation of Banti's concept. Thompson states "It is our current concept that Banti's disease or splenic anemia is the result of mechanical obstruction to the flow of blood within the portal system. With high portal pressure and relatively low peripheral pressure a reversal in the direction of normal blood flow can take place. This reversal is aided by the absence of valves in the portal system. A careful study of our clinical material leads to the conclusion that there are no significant differences clinically or hematologically between the congestive splenomegalies resulting from the various obstructive factors."
The histologie characteristics of the removed spleens were the same in all groups. All presented variable degrees of follicular atrophy, of fibrosis of the pulp with dilated venous sinuses, and all had the characteristic perifollicular hemorrhages. The obstructive lesions are either intrahepatic or extrahepatic. The intrahepatic lesion is cirrhosis; indeed, hepatic cirrhosis was the obstructive factor in 68 per cent of the cases. Rousselot and Thompson injected silica particles into the splenic veins of dogs and produced after two years a congestive splenomegaly with extensive collateral circulation. Thompson emphasizes that absence of cirrhosis as the obstructive agent at the time of splenectomy means that it will not appear subsequently. Thus he says "We have yet to see a patient develop the sequence of events characterized by Banti as the three stages of the syndrome."
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,9 on the basis of his extensive experience with schistosomiasis in the Orient, expresses the belief that most of the so-called Banti's disease of the Orient is actually schistosomiasis. He considers it probable that the disease as originally described by Banti might be schistosomiasis because of the proximity of Italy to Egypt, where the "Egyptian splenomegaly" has come to be recognized as schistosomial in origin.