Clinical analyses of 379 Japanese and 2 non-Japanese patients with venous or arterial vascular diseases: combined B cell anomalies, gastric cancer, and aggravated ectopic splenosis

Abstract

To investigate the increased expression of activation-induced cytidine deaminase (AID) in vascular diseases, 379 Japanese and 2 non-Japanese composed of 87 varix, 81 arteriosclerosis obliterans (ASO), 206 aortic aneurysm (AA), and 7 venous thrombosis (VT) patients, whose ages were ranged from 60.7 ± 13.3 to 74.5 ± 8.8 years old, were analyzed clinically. Their coagulation anomalies were described well using their levels of thrombin antithrombin III complex (TAT), α2-plasmin inhibitor-plasmin complex (PIC), and fibrin/fibrinogen degradation products-D dimer (FDP-DD) in all the 4 diseases. VT showed the most severe coagulopathy with the highest levels of TAT, PIC, and FDP-DD, followed by AA. Among the seven VT cases, a lympho-plasmacytic hypophysitis (IgG4-related hypophysitis) case and a retroperitoneal fibrosis case combined with malignant lymphoma (ML) were found. Another VT case involving portal vein thrombosis (PVT) demonstrated a low level (59%) of protein C antigen (Ag) together with ulcerative colitis. In the 81 ASO, two showed ML combined with gastric cancer (GC). Cancers were examined in 87 varix, 81 ASO, and 199 AA cases. The frequency of cancer was 9% (33/367), in which five cases showed double cancer. The most common cancer was gut cancers which were found in 5% cases (20/367). GC was found in 14 cases, and intestinal cancers were found in six cases. The AA onset of ten GC cases seemed to occur at the same time as the onset of GC, 60–70% of which showed postoperative serum amylase elevation. Ectopic splenosis aggravated the clinical symptoms in three AA patients who had undergone gastrectomy, cholecystectomy, or hepatectomy. Bleeding (hemolysis) and thrombosis were main trigger signs in the three AA patients. One of the three developed PVT. Existing high levels of activation-induced cytidine deaminase (AID) were judged from severe coagulopathy, combined B cell malignancy, accompanied autoimmunity, the highest frequency of GC, and aggravated splenosis. AID downregulation was concluded to be an important factor in order to stop vascular disease progressions.