Hepatic nodular lymphoid lesion with increased IgG4-positive plasma cells associated with primary biliary cirrhosis: a report of two cases

Abstract

The nodular lymphoid lesion of the liver known as reactive lymphoid hyperplasia or pseudolymphoma is rare and its pathogenesis is unknown. We report two cases of nodular lymphoid lesions of the liver with numerous IgG4-positive plasma cells in patients with primary biliary cirrhosis. Histologically, in both cases, the lesion showed a dense lymphoplasmacytic infiltrate with lymphoid follicles and granulomas. Fibrous tissue was scarce and without a storiform pattern. Obliterative phlebitis was not identified. The IgG4+ plasma cell counts were 82 and 76 per high power field, with an IgG4/IgG ratio of 75 and 64 %, respectively, which qualifies the lesions according to the diagnostic criteria for IgG4-related disease as « probable histological feature of IgG4-related disease ». There were no rearrangements of immunoglobulin heavy-chain genes and plasma cells had a polytypic pattern of kappa and lambda light-chain expression. The non-tumor liver showed primary biliary cirrhosis with destructive cholangitis without IgG4 plasma cells. In both cases, IgG4-related disease was not found in other organs neither at the time of diagnosis nor 3 years later. Serum IgG4 levels normalized after local ablation of the lesions. It seems unlikely that these lesions are a manifestation of IgG4-related disease. However, because the pathogenesis of both nodular lymphoid lesions and IgG4-related disease remains unclear, further studies are needed to elucidate a potential link between nodular lymphoid lesions of the liver and an increased number of IgG4 plasma cells. More definite conclusions will be possible when the pathogenesis of IgG4-related disease has been clarified.

A case of hepatic pseudolymphoma in a patient with primary biliary cirrhosis

“…In this case, HPL appeared during the course of PBC, but there were also 13 cases (16.3%) of HPL associated with PBC (Table ) . Because the lesion had been misdiagnosed as HCC or another malignant tumor, 10 HPL patients associated with PBC underwent hepatic resections.…”

“…Histologically, the interlobular bile ducts are primarily damaged and show characteristic findings, such as chronic nonsuppurative destructive cholangitis (CNSDC), followed by progressive bile duct loss . Some of the HPL patients present with extrahepatic autoimmune diseases, such as Sjogren's syndrome, autoimmune thyroiditis, Takayasu aortitis with antiphospholipid syndrome, or calcinosis, Raynaud's phenomenon, esophageal dysmotility, sclerodactyly, and telangiectasia (CREST syndrome; 13). Because HPL has a relatively large number of autoimmune disease complications, it is suggested that an autoimmune mechanism is involved in the increase in HPL .…”

Hepatic Pseudolymphoma with Fluorodeoxyglucose Uptake on Positron Emission Tomography

“…Recently, some reports have examined the use of FDG-PET in the diagnosis of hepatic pseudolymphoma. We found 9 cases in which FDG-PET was used to examine patients with hepatic pseudolymphoma, including our own (Table 2 ) [ 6 , 7 , 8 , 9 , 10 , 11 , 12 ]. Seven (78%) of 9 cases showed FDG uptake in the tumor, and the median SUV max was 4.3 (range: 3.4–7.2).…”

“…The preoperative diagnosis of hepatic pseudolymphoma is difficult because noninvasive radiological examinations such as ultrasonography (US), computed tomography (CT), and magnetic resonance imaging are usually equivocal. Recently, some reports have examined the use of fluorodeoxyglucose positron emission tomography (FDG-PET) in the diagnosis of hepatic pseudolymphoma [ 6 , 7 , 8 , 9 , 10 , 11 , 12 ]. We herein report a case of hepatic pseudolymphoma with FDG uptake on PET and examine the literature.…”

Contrast‐Enhanced Ultrasound Features of Hepatic Reactive Lymphoid Hyperplasia: Correlation With Histopathologic Findings

“…Histopathologically, it is characterized by a marked proliferation of non‐neoplastic polyclonal lymphocytes without atypia and mitosis, in which lymphoid follicles of various sizes and shapes with germinal centers can be detected . Until now, no more than 100 cases have been reported in the English literature . Follow‐up or a conservative strategy should be undertaken instead of surgery if a definite diagnosis is made .…”