Hemophagocytic syndrome in a child with severe Crohn's disease and familial Mediterranean fever.
Hemophagocytic lymphohistiocytosis (HLH) is a rare, potentially fatal, severe condition of hyperinflammation caused by the uncontrolled proliferation of activated lymphocytes and histiocytes secreting high amounts of inflammatory cytokines. Here we report a fatal hemophagocytic syndrome in a 11-year-old boy with a diagnosis of both Crohn's disease receiving immunosuppressive therapy and familial Mediterranean fever. It is important to evaluate the patients with inflammatory bowel disease receiving immunosuppressive therapy presenting with unexplained fever, cytopenia, progression of organomegaly and biochemical changes for the investigation of HLH for diagnosis and treatment.
Hacettepe University, Faculty of Medicine, Department of Pediatrics, Section of Gastroenterology, Hepatology and Nutrition, Ankara, Turkey. nurayu@hacettepe.edu.tr
Nuray
N
Uslu
Hulya
H
Demir
Gunay
G
Balta
Inci N
IN
Saltik-Temizel
Hasan
H
Ozen
Figen
F
Gürakan
Aysel
A
Yüce
D002648:Child; D003424:Crohn Disease / Q000150:complications / Q000188:drug therapy; D010505:Familial Mediterranean Fever / Q000150:complications* / Q000188:drug therapy; D017809:Fatal Outcome; D006801:Humans; D007165:Immunosuppression Therapy / Q000009:adverse effects; D007166:Immunosuppressive Agents / Q000009:adverse effects; D051359:Lymphohistiocytosis, Hemophagocytic / Q000150:complications / Q000175:diagnosis* / Q000188:drug therapy / Q000276:immunology; D008297:Male
D007166:Immunosuppressive Agents