Risk of Lymphoma in Primary Biliary Cirrhosis

Panjala, Chakri; Talwalkar, Jayant A.; Lindor, Keith D.

doi:10.1016/j.cgh.2007.02.020

Cited by 13 publications

(18 citation statements)

References 24 publications

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“…A potential association between lymphoma and PBC is suggested on the basis of individual reports in the literature [24][25][26][27][28][29] . In a retrospective study by Panjala et al [30] based on an estimated 2,912 patients evaluated at their institution during a 22-year period, only 13 (an estimated 0.6%) patients were evaluated in referral visits for evidence of lymphoma. Although we could find one case of transformation of hepatic pseudolymphoma into lymphoma [31] , we were unable to deduce the long term natural course of hepatic pseudolymphoma since most reported cases of hepatic lymphoma underwent surgical resection.…”

Section: Discussionmentioning

confidence: 99%

Pseudolymphoma of the liver associated with primary biliary cirrhosis: A case report and review of literature

Okada¹,

Mibayashi²,

Hasatani³

et al. 2009

WJG

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Section: Discussionmentioning

confidence: 99%

Pseudolymphoma of the liver associated with primary biliary cirrhosis: A case report and review of literature

Okada¹,

Mibayashi²,

Hasatani³

et al. 2009

WJG

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“…There are also several case reports linking PBC with NHL especially of the primary hepatic type [20–22]. However, a recent retrospective study by Panjala et al [23] failed to confirm such an association since the estimated baseline risk of lymphoma in 2912 patients with PBC was as low as <1%. Of note, the latter study showed that disease involvement was predominately extrahepatic, above the diaphragm [23].…”

Section: Discussionmentioning

confidence: 99%

Multiple Autoimmune Propensity and B-Non-Hodgkin Lymphoma: Cause or Effect?

Koumati

Palassopoulou

Matsouka

et al. 2011

Autoimmune Diseases

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We report a case of multiple autoimmunity consisting of the presence of autoimmune haemolytic anaemia (AIHA), antimitochondrial antibodies (AMAs), and antiphospholipid antibodies (APLAbs) as the presenting manifestations of an extrahepatic B-non-Hodgkin lymphoma (B-NHL) in a 63-year-old woman. The patient presented with fatigue attributed to severe AIHA. Due to increased serum IgM and γ-GT levels, an investigation for AMA was performed, which proved positive with anti-M2 specificity. A prolongation of activated partial thromboplastin time (aPTT) led to the determination of APLAbs (lupus anticoagulant and other APLAbs) which were also positive. Bone marrow biopsy in combination with immmunohistochemical studies established the diagnosis of lymphoplasmacytic B-NHL. Ten months later, B-NHL was in remission while AMA and APLAbs were still positive. In conclusion, we documented the coexistence of multiple autoimmune reactions together with B-NHL highlighting the possible common pathogenetic pathways of the two entities.

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“…The risk for lymphoma in patients with PBC is negligible. Panjala et al demonstrated that in 2,193 PBC patients only 13 cases were diagnosed with lymphoma [1].…”

Section: Image Of the Issuementioning

confidence: 99%

“…The risk for lymphoma in patients with PBC is negligible. Panjala et al demonstrated that in 2,193 PBC patients only 13 cases were diagnosed with lymphoma [1].The short survival of NHL with major liver involvement makes early diagnosis essential [2]. Due to better imaging, molecular and serological tests and the concern for the development of port-side metastases [3], less biopsies are performed.…”

mentioning

confidence: 99%

Malignancy Presenting as Multiple Lesions in a Cirrhotic Liver: Not Always Hepatocellular Carcinoma

Deure

Lugtenburg

Dwarkasing

et al. 2020

JGLD

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A 65-year-old female with primary biliary cirrhosis (PBC) presented with four hepatic lesions, visualized on a routinely performed ultrasound. She had no complaints. Clinical examination was normal. Laboratory investigation showed elevated liver tests, with a bilirubin level of 21 IU/l (normal <16 IU/l) and gamma-glutamyltransferase of 222 IU/l (normal < 37U/l). Alfa-fetoprotein was 3μg/l (normal < 9μg/l). Magnetic resonance imaging of the abdomen showed cirrhosis of the liver with lesions in segments II, III, IV and VIII. The lesions demonstrated high signal intensity on T2-weighted ( Fig. 1A) and diffusion imaging (Fig. 1B), including enhancement in the arterial phase after intravenous gadolinium administration, which raised suspicion of multifocal hepatocellular carcinoma. An extended left hepatectomy and radio frequency ablation were scheduled. During the laparotomy multiple lesions in the right liver and the ligamentum hepatoduodenale were found. Histology showed liver tissue with cirrhotic nodules and lymphatic lesions with populations of BCL2 positive B-cells, compatible with a marginal zone B-cell lymphoma (Fig. 1C). Staging showed an identical lymphoma in the bone marrow, compatible with stage IV non-Hodgkin's lymphoma (NHL). The patient was not responsive to immuno-chemotherapy and died after several weeks.Hepatic involvement of NHL can cause an elevation of liverassociated enzymes due to diffuse infiltration, but typically does not result in hepatic dysfunction and very rarely manifests as circumscribed lesions. Therefore, NHL is often misdiagnosed as primary liver cancer, metastases, focal nodular hyperplasia IMAGE OF THE ISSUEor abscesses. The risk for lymphoma in patients with PBC is negligible. Panjala et al. demonstrated that in 2,193 PBC patients only 13 cases were diagnosed with lymphoma [1].The short survival of NHL with major liver involvement makes early diagnosis essential [2]. Due to better imaging, molecular and serological tests and the concern for the development of port-side metastases [3], less biopsies are performed. If the clinical picture is suspicious for NHL, a liver biopsy should be attempted.

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Risk of Lymphoma in Primary Biliary Cirrhosis

Cited by 13 publications

References 24 publications

Pseudolymphoma of the liver associated with primary biliary cirrhosis: A case report and review of literature

Pseudolymphoma of the liver associated with primary biliary cirrhosis: A case report and review of literature

Multiple Autoimmune Propensity and B-Non-Hodgkin Lymphoma: Cause or Effect?

Malignancy Presenting as Multiple Lesions in a Cirrhotic Liver: Not Always Hepatocellular Carcinoma

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