OBJECTIVE: To assess the incidence of hemophagocytic lymphohistiocytosis (HLH) in a well-defined population of children with inflammatory bowel disease (IBD) and evaluate the common clinical and laboratory characteristics of individuals with IBD who developed HLH. STUDY DESIGN: We conducted a retrospective study of all children who developed HLH over an 8-year period. The incidence of HLH in patients with IBD was calculated using US census data and a statewide project examining the epidemiology of pediatric IBD. RESULTS: Among children in Wisconsin, 20 cases of HLH occurred during the study period; 5 cases occurred in children with IBD. Common characteristics include: Crohn's disease (CD), thiopurine administration, fever lasting more than 5 days, lymphadenopathy, splenomegaly, anemia, lymphopenia, and elevated serum triglycerides and ferritin. Of the patients, 4 had primary Epstein-Barr virus infections. The incidence of HLH among all children in Wisconsin was 1.5 per 100 000 per year. The risk was more than 100-fold greater for children with CD (P < .00001). CONCLUSIONS: Pediatric patients with CD are at increased risk for developing HLH; primary Epstein-Barr virus infection and thiopurine administration may be risk factors.
OBJECTIVE: To assess the incidence of hemophagocytic lymphohistiocytosis (HLH) in a well-defined population of children with inflammatory bowel disease (IBD) and evaluate the common clinical and laboratory characteristics of individuals with IBD who developed HLH. STUDY DESIGN: We conducted a retrospective study of all children who developed HLH over an 8-year period. The incidence of HLH in patients with IBD was calculated using US census data and a statewide project examining the epidemiology of pediatric IBD. RESULTS: Among children in Wisconsin, 20 cases of HLH occurred during the study period; 5 cases occurred in children with IBD. Common characteristics include: Crohn's disease (CD), thiopurine administration, fever lasting more than 5 days, lymphadenopathy, splenomegaly, anemia, lymphopenia, and elevated serum triglycerides and ferritin. Of the patients, 4 had primary Epstein-Barr virus infections. The incidence of HLH among all children in Wisconsin was 1.5 per 100 000 per year. The risk was more than 100-fold greater for children with CD (P < .00001). CONCLUSIONS: Pediatric patients with CD are at increased risk for developing HLH; primary Epstein-Barr virus infection and thiopurine administration may be risk factors.
Authors: Eric Chauveau; François Terrier; Didier Casassus-Buihle; Xavier Moncoucy; Bernard Oddes Journal: Presse Med Date: 2005-04-23 Impact factor: 1.228
Authors: Y W Sijpkens; C F Allaart; J Thompson; J van't Wout; P M Kluin; G J den Ottolander; R Bieger Journal: Ann Hematol Date: 1996-04 Impact factor: 3.673
Authors: J I Henter; M Aricò; R M Egeler; G Elinder; B E Favara; A H Filipovich; H Gadner; S Imashuku; G Janka-Schaub; D Komp; S Ladisch; D Webb Journal: Med Pediatr Oncol Date: 1997-05
Authors: Andrew Wisniewski; Julien Kirchgesner; Philippe Seksik; Cécilia Landman; Anne Bourrier; Isabelle Nion-Larmurier; Philippe Marteau; Jacques Cosnes; Harry Sokol; Laurent Beaugerie Journal: United European Gastroenterol J Date: 2019-11-14 Impact factor: 4.623