A rare cause of acute liver failure due to haemophagocytic lymphohistiocytosis secondary to diffuse large B-cell lymphoma.

Acute liver failure is a life-threatening condition commonly caused by drug-induced hepatotoxicity or viral hepatitides. However, there are a number of rarer causes such as haemophagocytic lymphohistiocytosis. Haemophagocytic lymphohistiocytosis is a syndrome of uncontrolled immune cell activation, triggered by infection or malignancy, which carries a high mortality. Whilst mild to moderate liver injury is commonly seen with haemophagocytic lymphohistiocytosis, acute liver failure has rarely been reported in adults. We present a case of a 74-year-old man with acute liver failure secondary to haemophagocytic lymphohistiocytosis triggered by undiagnosed large B-cell lymphoma. Initially treated for biliary sepsis, there was a delay in the diagnosis of haemophagocytic lymphohistiocytosis and despite initiating chemotherapy, he died soon after. This case highlights the importance of considering haemophagocytic lymphohistiocytosis as a rare cause of acute liver failure, as given the life-threatening potential of haemophagocytic lymphohistiocytosis, a prompt diagnosis may allow early initiation of chemotherapy for any chance of survival.

Outcomes after ileal pouch anal anastomosis in patients with primary sclerosing cholangitis.

BACKGROUND AND AIMS: Outcomes after ileal pouch anal anastomosis (IPAA) are not well established in patients with primary sclerosing cholangitis (PSC). We conducted a comprehensive outcomes assessment in these patients. METHODS: A retrospective case note review of complications in all PSC-IPAA (n=21) and matched ulcerative colitis patients with IPAA (UC-IPAA; n=79) after surgery in Oxford (1983-2012) was conducted, and functional outcomes (Öresland score) were evaluated (2012). Quality of life [Cleveland Global Quality of Life Questionnaire, Short Form-36 (SF-36)], and sexual function were also assessed (2012) including patients with PSC-associated UC without IPAA (PSC-UC; n=19). Sub-group analysis of patients with large duct (ld) PSC-IPAA (n=17) was also performed. RESULTS: The 1-, 5-, 10- and 20-year risk of acute pouchitis for PSC-IPAA was 10%, 19%, 31% and 65% respectively, compared to 3%, 10%, 14% and 28% in UC-IPAA (p=0.03). More PSC-IPAA (36%) had poor nocturnal pouch function (vs 2% in UC-IPAA; p=0.0016). There were no differences in surgical complications, quality of life or sexual function between the 3 main groups. LdPSC-IPAA had poorer pouch function (Öresland score: 7.7 vs 5.4 in UC-IPAA; p=0.02), and worse quality of life [SF-36 Physical: 42 vs 50.5 in UC-IPAA; 47.7 in PSC-UC; p=0.03 and Mental Health summary scores: 41.6 vs 51.2 in UC-IPAA; 42.3 in PSC-UC; p=0.04]. CONCLUSIONS: PSC-IPAA suffer more acute pouchitis and have worse functional outcomes than UC-IPAA. LdPSC-IPAA also have poorer quality of life.

Cholangiocarcinoma complicating secondary sclerosing cholangitis from cryptosporidiosis in an adult patient with CD40 ligand deficiency: case report and review of the literature.

A 43-year-old man with a hyper-immunoglobulin M syndrome due to CD40 ligand deficiency presented with insidious onset of recurrent diarrhoea and deranged liver function tests. Standard stool microscopy was repeatedly negative for cryptosporidia but immunofluorescent testing and polymerase chain reaction demonstrated the presence of infection eventually. Despite both paromomycin and nitazoxanide, he developed sclerosing cholangitis secondary to cryptosporidial infection. Whilst being considered for dual bone marrow and liver transplantation, he was found to have cholangiocarcinoma on imaging after three biopsies of a suspicious lesion. This is a rare complication of this combined immune deficiency predominantly in children that has not been reported previously in a long-term survivor with this condition.

The clinical impact of primary sclerosing cholangitis in patients with an ileal pouch-anal anastomosis for ulcerative colitis.

INTRODUCTION: Primary sclerosing cholangitis (PSC), a chronic inflammatory disease affecting the liver, is associated with ulcerative colitis (UC) in up to 75% of Northern European patients. These patients are at increased risk for the development of colorectal cancer, and the operation of choice is restorative proctocolectomy with an ileal pouch-anal anastomosis. However, complications such as pouchitis can occur, and studies have suggested that PSC is an independent risk factor for the development of pouchitis. AIM: The aim of this study is to review and discuss the available literature on the effect of PSC on clinical outcomes of patients who undergo pouch surgery for UC. The outcomes reviewed comprise the incidence of pouchitis and pouch dysplasia/cancer and quality of life, including sexual function in UC patients with or without PSC. METHODS: Pubmed/Medline and Embase searches were undertaken to obtain papers in English between 1966 and 2008. The keywords used were primary sclerosing cholangitis, ulcerative colitis, ileal pouch-anal anastomosis, quality of life, sexual function, dysplasia or cancer, pouchitis and orthotopic liver transplantation. RESULTS: The incidence of pouchitis, pouch mucosal atrophy and risk of dysplasia appear to be greater in patients with associated PSC than in UC patients without PSC. Quality of life does not appear to be worse than in patients without PSC. Sexual function has not been studied in this subgroup of patients. CONCLUSION: Pouchitis appears to be more common in the subset of UC patients with PSC, although there is clearly a need for further well-designed studies.