Primary sclerosing cholangitis and Hodgkin’s disease

Man, Kwun Nok Mimi; Drejet, Anne; Keeffe, Emmet B.; Garcia-Kennedy, Richard; Imperial, Joanne C.; Esquivel, Carlos O.

doi:10.1002/hep.1840180518

Cited by 28 publications

(5 citation statements)

References 26 publications

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“…There is one candidate gene study in non-Hodgkin lymphoma suggesting an association with SNPs in the BCL2L11 gene (rs3789068 in particular). 91 Interestingly, there are several case reports on the occurrence of non-Hodgkin as well as Hodgkin lymphoma in PSC, 92,93 but an association is not formally established. The lead PSC-associated SNP (rs6720394) is located downstream of the BCL2L11 gene, and due to a lack of functional studies cannot be concluded causal.…”

Section: Bcl2l11 (Bim)mentioning

confidence: 99%

Deciphering the Genetic Predisposition to Primary Sclerosing Cholangitis

Karlsen

Kaser²

2011

Semin Liver Dis

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Genetic variants within the major histocompatibility complex (MHC) on chromosome 6 have been shown to confer risk for primary sclerosing cholangitis (PSC) ~30 years ago. However, robust genetic associations outside this genetic region have been difficult to establish. By genome-wide association analysis, a surprising large overlap of genetic risk loci outside of the MHC with prototypical autoimmune diseases has been recognized. In this article, we review the present knowledge of susceptibility loci in PSC, by assessing the robustness of the findings and speculating on potential mechanistic roles of predicted risk genes in PSC pathogenesis. We suggest a model where the primary insult is likely to resemble the tissue injury in most autoimmune conditions. Functional insight into risk pathways could offer novel therapeutic opportunities, and we speculate on specific opportunities that may arise based on current knowledge.

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Section: Bcl2l11 (Bim)mentioning

confidence: 99%

Deciphering the Genetic Predisposition to Primary Sclerosing Cholangitis

Karlsen

Kaser²

2011

Semin Liver Dis

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“…According to one report, sclerosing cholangitis predated the diagnosis of Hodgkin's disease by 2, 11, and 17 years in three patients who were over the age of 40 years when the latter condition developed. 21 The diagnosis in this case is probably sclerosing cholangitis. A sweat test should be performed to rule out cystic fibrosis.…”

mentioning

confidence: 78%

Case 19-2000

Katz¹,

Graeme‐Cook²

2000

N Engl J Med

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A 17-year-old boy was admitted to the hospital because of obstructive jaundice and weight loss.The patient had been well until two months earlier, when he consulted a physician because of pruritus. Topical antibiotic preparations were prescribed for excoriations, without improvement. Laboratory studies were performed a week later and were repeated after seven days (Table 1). An abdominal ultrasonographic examination showed distention of the gallbladder and common bile duct, with two large cysts in the left upper quadrant. Three days before admission, the patient came to this hospital.The patient had been born in the United States; his parents were Haitian immigrants. He had lost 13 kg in weight during the preceding three months, during which he had begun to run up to 1500 m daily. His stools became lighter and his urine darker several weeks before admission. He had no history of trauma, alcohol abuse, or use of illicit drugs and no risk factors for human immunodeficiency virus (HIV) infection. His parents and seven siblings were well. There was no family history of disorders suggestive of pancreaticobiliary disease.The temperature was 37°C, the pulse was 63, and the respirations were 18. The blood pressure was 120/65 mm Hg, and the weight was 74 kg.Physical examination showed no abnormalities except for excoriations and scleral icterus.Laboratory studies were performed (Tables 1 and 2). An axial T 2 -weighted image from a magnetic resonance imaging (MRI) study (Fig. 1) showed two homogeneous cysts adjacent to the tail of the pancreas. A magnetic resonance cholangiopancreatographic study (Fig. 2) showed a focal stricture in the common bile duct, which terminated at an abnormally high level; an *To convert the values for bilirubin to micromoles per liter, multiply by 17.1. †The normal range is 5 to 50 U per liter.

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“…While liver diseases occur as a hepatic manifestation of paraneoplastic syndrome in HL [ 47 – 49 ], the frequency of direct involvement with HL ranges from 2% to 20% at the time of diagnosis [ 1 , 50 , 51 ] and up to two-thirds of the cases at autopsy or necropsy [ 15 , 52 ]. The portal tracts are the most common site for HL involvement [ 52 ].…”

Section: Discussionmentioning

confidence: 99%

An Autopsy Case of an Elderly Patient with Classic Hodgkin Lymphoma Presenting with a Plethora of Clinical Symptoms and Signs

et al. 2020

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Patient: Female, 88-year-old Final Diagnosis: Hodgkin lymphoma Symptoms: Fever • weight loss • dypnea Medication: — Clinical Procedure: — Specialty: Pathology Objective: Unusual clinical course Background: Hodgkin lymphoma (HL) is a potentially curable disease with favorable outcomes. However, elderly patients with HL usually have more adverse prognostic factors and hence a much worse prognosis than younger patients. Case Report: The patient was a woman in her 80s. She reported high fever, anorexia, and a weight loss of 8 kg within 5 months. She had been on treatment for diabetes mellitus and hypertension. She had undergone percutaneous coronary intervention and pacemaker implantation to treat acute coronary syndrome and sinus arrhythmia, respectively. Blood tests showed elevation of alkaline phosphatase, C-reactive protein, leukocyte count, CA 19-9, and carcinoembryonic antigen. Computed tomography did not show tumors in the liver, and cholangitis and sepsis were suspected. Aspartate transaminase, alanine aminotransferase, and total bilirubin gradually increased through the course of the patient’s hospital stay. Despite treatment, her condition deteriorated and she died 22 days after hospital admission. At autopsy, we found stage IV HL with lymph node swelling on both sides of the diaphragm, as well as diffusely disseminated nodules in the liver and spleen. Conclusions: Our patient had several poor prognostic factors including B symptoms, comorbidity, advanced stage, Epstein-Barr virus infection, and expression of programmed death-ligand 1 and interleukin-6, all of which were closely connected with her advanced age. Her age and comorbidities may have been the most adverse prognostic factors for her illness. An effective HL screening method for elderly individuals should be developed to ameliorate poor prognosis and adverse outcomes.

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Primary sclerosing cholangitis and Hodgkin’s disease

Cited by 28 publications

References 26 publications

Deciphering the Genetic Predisposition to Primary Sclerosing Cholangitis

Deciphering the Genetic Predisposition to Primary Sclerosing Cholangitis

Case 19-2000

An Autopsy Case of an Elderly Patient with Classic Hodgkin Lymphoma Presenting with a Plethora of Clinical Symptoms and Signs

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