Intravascular large
B-cell lymphoma (IVLBCL), which involves the lumen of small vessels, is a rare variant of extranodal diffuse large
B-cell lymphomas. Herein, we present a case of IVLBCL manifesting as
cholecystitis in a 77-year-old Japanese man. He presented with
fever,
fatigue, and
weight loss. Physical examination revealed tenderness of the right upper quadrant. The white blood cell count and
C-reactive protein levels were elevated. Computed tomography revealed gallbladder thickening and pericholecystic fluid collection; these observations were consistent with the diagnosis of
cholecystitis. Serum soluble
interleukin-2 receptor levels were highly elevated, and
gallium scintigraphy revealed an abnormal accumulation in the spleen, implying
lymphoma. Consequently, G-banding analysis of the patient's bone marrow aspirates revealed the presence of different abnormal clones, including those with gain of chromosome 18 and deletion of chromosome 6q. As
cholecystectomy was necessary, a concurrent
splenectomy was performed to diagnose the disease definitively. Histopathologically, atypical large lymphoid cells were observed to be localized in the vasculature in both the spleen and gallbladder; the atypical cells expressed high levels of CD20, CD5, and CD10, immunohistochemically. These findings were consistent with IVLBCL. The patient underwent post-operative treatment with
rituximab,
cyclophosphamide,
adriamycin,
vincristine, and
prednisolone. However, a
pancreatic fistula developed during
chemotherapy, causing left
pleural effusion and peritoneal effusion; the patient developed
sepsis from multidrug-resistant microorganisms, and subsequently died of multi-organ failure 6 months after the diagnosis. No obvious recurrence of the
tumor was found during autopsy. We discuss the characteristic karyotype and immunohistochemical status observed in this case.