Fever and pancytopenia in a patient with Crohn's disease

Weinkove, Robert; Dickson, Michelle; Eliadou, Elena; Stace, Nigel H.; Goossens, Louise; Ferguson, Peter M.

doi:10.1136/gutjnl-2012-303632

Cited by 3 publications

(4 citation statements)

References 4 publications

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“…We identified 50 cases of MAS associated with IBD including two unpublished cases from our clinic and one case from the IBD‐centre of Palermo (Table ),. The first report was published in 1988 followed by six isolated reports over the following 16 years, up to 2004.…”

Section: Resultsmentioning

confidence: 99%

“…When indicated, thiopurine dosing ranged from 0.9 to 3.15 mg/kg/day for azathioprine and 1.4 to 1.76 mg/kg/day for mercaptopurine. In six patients (Table ), determination of erythrocyte concentrations of 6‐thioguanine nucleotides (6‐TGN), and in four patients also those of 6‐methylmercaptopurines (6‐MMP), was available shortly before onset of MAS, showing the former levels to be within the therapeutic range and the latter below the toxic limit …”

Section: Resultsmentioning

confidence: 99%

“…In six patients (Table 3), determination of erythrocyte concentrations of 6-thioguanine nucleotides (6-TGN), and in four patients also those of 6-methylmercaptopurines (6-MMP), was available shortly before onset of MAS, showing the former levels to be within the therapeutic range and the latter below the toxic limit. 27,44,50,51 Mesalazine or sulphasalazine alone were reported in one patient, steroids alone in two patients and steroids followed by colectomy in one patient with UC.…”

Section: Resultsmentioning

confidence: 99%

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Systematic review: macrophage activation syndrome in inflammatory bowel disease

Fries

Cottone

Cascio

2013

Aliment Pharmacol Ther

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Section: Resultsmentioning

confidence: 99%

Section: Resultsmentioning

confidence: 99%

Section: Resultsmentioning

confidence: 99%

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Systematic review: macrophage activation syndrome in inflammatory bowel disease

Fries

Cottone

Cascio

2013

Aliment Pharmacol Ther

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“…Haemophagocytic lymphohistiocytosis is a clinical syndrome of excessive macrophage activation 7 which rarely causes acute liver failure. Herein we have presented a case of acute liver failure secondary to haemophagocytic lymphohistiocytosis due to an undiagnosed large B-cell lymphoma in an elderly gentleman.…”

Section: Discussionmentioning

confidence: 99%

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

et al. 2021

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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.

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Hemophagocytic Lymphohistiocytosis and Inflammatory Bowel Disease: Case Report and Systematic Review

et al. 2020

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Aim: To report a case of a female patient with hemophagocytic lymphohistiocytosis (HLH) and to systematically review the available cases of the association between HLH and inflammatory bowel disease (IBD). Methods: In accordance to Preferred Reporting Items for Systematic Reviews and Meta-Analysis guidelines, retrieval of studies was based on Medical Subject Headings and Health Sciences Descriptors, which were combined using Boolean operators. Searches were run on the electronic databases Scopus, Web of Science, MED-LINE (PubMed), Biblioteca Regional de Medicina, Latin American and Caribbean Health Sciences Literature, Cochrane Library for Systematic Reviews and Opengrey.eu. Languages were restricted to English, Spanish and Portuguese. There was no date of publication restrictions. The reference lists of the studies retrieved were searched manually. Results: The search strategy retrieved 223 references. In the final analysis, 28 references were included, with the report of 35 cases. The most common clinical finding was fever, 57% of patients had a cytomegalovirus infection and 30 patients were on thiopurines previously to HLH diagnosis. Most patients were treated with steroids and antiviral therapy. All-cause mortality was 22%. Conclusion: These findings suggest that there might be a connection of HLH to IBD, opportunistic viral infections and the use of thiopurines. Due to the severity of such disease, the clinical suspicion is paramount to early diagnosis and therapy.

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Fever and pancytopenia in a patient with Crohn's disease

Cited by 3 publications

References 4 publications

Systematic review: macrophage activation syndrome in inflammatory bowel disease

Systematic review: macrophage activation syndrome in inflammatory bowel disease

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

Hemophagocytic Lymphohistiocytosis and Inflammatory Bowel Disease: Case Report and Systematic Review

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