Research Day
LANGERHANS CELL HISTIOCYTOSIS (LCH) OF GI TRACT IN A 6-MONTH-OLD FEMALE
Document Type
Abstract
Date
2021
Abstract
Introduction: Langerhans cell histiocytosis (LCH) is a rare hematologic disorder involving a proliferation of myeloid progenitor cells resembling dendritic immune Langerhans cells. The disease has a wide variety of clinical presentations with various local and systemic involvements. We present a case of a 6-month-old diagnosed with LCH presenting with GI tract involvement.
Case History: The patient is a 6-month-old female with severe malnutrition, lifelong feeding intolerance and recurrent bloody stools who presented with two days of bilious emesis and increasing feeding intolerance. Laboratory workup notable for hyponatremia, elevated LFTs, severe hypoalbuminemia, normocytic anemia, and leukocytosis with elevated inflammatory markers. Imaging demonstrated hepatomegaly with parenchymal edema and steatosis. A narrow proximal small bowel was seen on small bowel follow-through. EGD was significant for diffuse ulceration of the duodenum and congested mucosa in the stomach and rectum. Biopsy confirmed LCH in the stomach and recto-sigmoid colon. Bone marrow biopsy was positive for BRAF by PCR. After diagnosis, patient was started on monotherapy with cytarabine for treatment of systemic LCH.
Conclusions: LCH is a rare condition, and clinical presentation is highly variable. Involvement of GI tract is uncommon and is often indicative of systemic disease, which requires more aggressive treatment. As in this patient, LCH can mimic milk protein allergy, but it does not improve with dietary modifications or age. Although rare, LCH should be considered in infants with hematochezia and failure to thrive in the presence of feeding intolerance.