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Boletín médico del Hospital Infantil de México
Print version ISSN 1665-1146
Abstract
CHAVEZ-AGUILAR, Lénica A.; FLORES-VILLEGAS, Victoria and ZARAGOZA-AREVALO, Gerardo R.. Hemophagocytic lymphohistiocytosis as a presentation of inflammatory bowel disease. Bol. Med. Hosp. Infant. Mex. [online]. 2020, vol.77, n.4, pp.207-211. Epub Sep 29, 2020. ISSN 1665-1146. https://doi.org/10.24875/bmhim.20000013.
Background:
Hemophagocytic lymphohistiocytosis (HLH) is considered a medical emergency that should be recognized in patients with fever, splenomegaly, and progressive deterioration of the general condition. Laboratory findings include cytopenia, hypertriglyceridemia, hypofibrinogenemia, and hyperferritinemia. For HLH diagnosis, it is essential, although not mandatory, to perform a bone marrow biopsy. Given its nature, secondary causes of HLH should be sought, mainly infections, hemato-oncological disorders, autoimmune diseases, and auto-inflammatory conditions.
Case report:
We present the case of a female adolescent who presented with fever and lower gastrointestinal bleeding. Upon admission, acute liver failure and pancytopenia were documented. A bone marrow aspirate was performed, which revealed hemophagocytosis; other tests confirmed HLH diagnosis. During the diagnostic approach, inflammatory bowel disease was diagnosed. The patient received first-line treatment with an adequate response.
Conclusions:
Inflammatory bowel disease can be considered a cause of secondary HLH, particularly in patients with suggestive symptoms, such as digestive bleeding in the absence of other secondary causes of HLH.
Keywords : Hemophagocytic lymphohistiocytosis; Inflammatory bowel disease; Ulcerative colitis.