To Örebro University

BACKGROUND: Idiopathic Thrombocytopenic Purpura (ITP) is an acquired haematological disorder with an incidence of 1 to 6 per 100,000/year. ITP and inflammatory bowel disease (IBD) comorbidity has been reported in the literature, but insights regarding the course, outcome and optimal management are limited by its rarity. The current study aimed to evaluate the clinical presentation and outcome of ITP in patients with IBD.

METHODS: This multicentre retrospective case series was performed as part of the ECCO Collaborative Network of Exceptionally Rare case reports (CONFER) project. Cases of patients with ITP and IBD were collected by participating investigators. Clinical data were recorded in a standardised collection form.

RESULTS: This report includes 32 patients with concurrent ITP and IBD:10 were females, median age was 32.0 [interquartile range (IQR) 20.5-39.5]. 14 patients had a diagnosis of Crohn's disease (CD) and the other 18 had of ulcerative colitis (UC). The diagnosis of IBD preceded the ITP in 26 patients (median time between diagnoses was 7.0 years [IQR, 1.5-9.5]). Among those patients, 17 patients were in clinical remission at ITP diagnosis. 13 patients were treated with mesalamine, 4 with oral corticosteroids, 1 with rectal corticosteroids, 2 with azathioprine, and 5 with anti-TNF agents. The median platelet count was 35,000/mmc (IQR, 10,000-70,000). 8 patients had rectal bleeding, 13 had skin purpura, 3 had epistaxis, 6 had mucosal petechiae, and 13 were asymptomatic. Regarding ITP treatment, 19 were treated with corticosteroids, 1 with Anti-RhD immunoglobulin, 12 with intravenous immunoglobulins (IVIG), 4 with thrombopoietin, 3 with rituximab and 6 patients eventually required splenectomy. 10 patients needed no treatment directed to the ITP.Three patients required colectomy during term long follow-up, due to IBD or cancer and not to massive bleeding as a complication of ITP. One patient of eight patients who presented with rectal bleeding required splenectomy, and none required urgent colectomy. Two patients died during the follow-up, one of them due to bleeding complication located in the upper gastrointestinal tract.Median follow-up time was 6.5 years [IQR, 3-10]. With long-term follow-up, all patients had platelet count above 50,000/mmc, and 24 were in IBD clinical remission.

CONCLUSION: Most ITP cases in this case series occurred after the IBD diagnosis and responded well to regular ITP treatment. The course of the ITP in the IBD patients follows an expected course, including response to medical therapy and low rates of splenectomy.