Hepatosplenic T cell lymphoma: a unifying entity in a patient with hemolytic anemia, massive splenomegaly, and liver dysfunction

Mavilia, Marianna G.; McAuliffe, Agnes; Hafeez, Safina; Vaziri, Haleh

doi:10.1007/s12328-018-0869-x

Cited by 2 publications

(1 citation statement)

References 23 publications

Supporting

Mentioning

Contrasting

Order By: Relevance

“…There are specific increases in risk of non‐melanotic skin cancer and lymphoproliferative cancers. Fatal hepatosplenic T cell lymphoma (HSTCL) has been reported in patients taking azathioprine for over 10 years . In IBD patients, the absolute risk of developing HSTCL on thiopurine monotherapy is 1:45000 in patients with IBD (and was higher in those under 35).…”

Section: Review Criteriamentioning

confidence: 99%

Stopping immunosuppressive treatment in autoimmune hepatitis (AIH): Is it justified (and in whom and when)?

Harrison

Gleeson

2019

Liver International

View full text Add to dashboard Cite

Background Initial treatment of autoimmune hepatitis (AIH) with prednisolone ± azathioprine is based on randomised controlled trials. Many patients receive long‐term immunosuppressive treatment to prevent disease relapse; this strategy has a weaker evidence base. Aim To consider whether immunosuppressive treatment (IST) withdrawal in AIH is justified and to develop a rationale for patient selection. Methods We reviewed published papers between 1972 and 2018, which addressed the outcomes of IST withdrawal and/or complications of IST in AIH. Results (1) AIH relapse rates after withdrawal of IST vary between 25% and 100%. There is heterogeneity in these studies regarding relapse definition, IST duration prior to withdrawal and criteria for biochemical and histological remission prior to withdrawal. (2) Factors associated with relapse following IST withdrawal include: (a) absence of an identifiable initial disease trigger, (b) presence of other autoimmune diseases, (c) longer time to biochemical remission and (d) elevated serum transaminases on treatment withdrawal. Reports of associations between relapse and age, IST duration and failure of histological remission have been inconsistent. (3) Continued IST reduces risk of AIH relapse over at least 5 years. However, there is no evidence that routine (as opposed to selective) long‐term IST improves disease outcome. (4) Patients with AIH have an increased risk of extrahepatic cancer, notably non‐melanoma skin cancer, to which long‐term IST may contribute. Long‐term corticosteroid therapy is associated with weight gain, low‐trauma fractures, diabetes and possibly vascular disease. Conclusions While further studies are needed, evidence supports a strategy of IST withdrawal in some patients with AIH who have achieved remission.

show abstract

Section: Review Criteriamentioning

confidence: 99%