Features of patients with inflammatory bowel diseases who develop hemophagocytic lymphohistiocytosis
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Hemophagocytic lymphohistiocytosis (HLH) is a serious hyperinflammatory condition that develops sporadically in patients undergoing treatment for inflammatory bowel disease (IBD). We collected data from a patient who developed HLH while undergoing treatment for IBD, as well as from 21 other cases through a literature search. We summarized clinical data, laboratory test results, imaging findings, and information on treatments and outcomes, and compared with the general IBD patients in two hospitals.
Altogether, 16 papers were collected. Most patients (20/22) were less than 45 years old, the male to female ratio was 12:10, and Crohn’s disease (CD):ulcerative colitis (UC) was 18:4. As for immunosuppressive regimen, nine patients were under thiopurines in monotherapy, in combination with steroids (n = 4) and infliximab (n = 4); three patients was on steroids monotherapy and one patient in combination with infliximab. Effectiveness of the immunosuppressive regimen was...