Florida State University, Sarasota Memorial Hospital Sarasota, FL, United States
Mindy Ward, DO1, Precious Idogun, MD2, Nathan Seepaulsing, 2, Richard Brown, MD2, Coppola Domenico, MD3, Scott Corbett, MD2 1Florida State University, Sarasota Memorial Hospital, Sarasota, FL; 2Florida State University, Sarasota, FL; 3Florida Digestive Health Specialists, Lakewood Ranch, FL
Introduction: Langerhans cell histiocytosis (LCH) is a rare myeloid neoplasm characterized by the overproduction and accumulation of CD1a+/Langerin+ LCH cells. Clinical manifestations are variable, ranging from localized disease to multi-system involvement. LCH is primarily described as a childhood illness with integumentary and skeletal involvement. Here, we present a rare case of an elderly adult LCH with extensive colonic, rectal and bony involvement diagnosed by colonoscopy.
Case Description/Methods: An 84-year-old woman with a past medical history of adenomatous colon polyps presents for a five-year surveillance colonoscopy. Prior to colonoscopy, she discussed a change in stool, describing 1-3 softer bowel movements in the morning and occasional discharge without any blood. Colonoscopy showed, two polyps, scattered erosions and ulcerations not typical of Crohn's disease or ulcerative colitis, and a single ulceration in the mid rectum that were all biopsied.
All biopsies returned showing ulceration and granulation tissue with histiocytic cells confirmed to be LCH, also showing positive BRAF mutation, S100, and CD1a.
Prior to returning for follow-up, the patient developed anaphylaxis to Lisinopril. During hospitalization she contracted clostridium difficile and received treatment. With few ongoing symptoms she was reluctant to undergo treatment for LCH. A repeat colonoscopy ten months later again confirmed LCH. The patient underwent imaging, identifying a bony lesion in the left rib. She went on to receive six courses of vinblastine.
Discussion: LCH is uncommon in adults. Multi-system disease with extensive gastrointestinal involvement of the colon and rectum is extremely rare, the disease is most commonly found in children. We present a rare case of colon, rectum, and bone involvement in an 84-year-old woman. This patient's case could easily have been mistaken for Crohn's disease or other colitis.
Treatment of multi-system disease in this age population is not standardized. Some cases have shown spontaneous remission; however, notably this patient still had extensive colonic disease ten months following her initial colonoscopy. Chemotherapy is often recommended; although chemo was delayed, she received six cycles of vinblastine with resolution of GI symptoms, pending further follow-up. Expert pathologic evaluation was critical in arriving at appropriate diagnosis in an unusual circumstance.
Mindy Ward indicated no relevant financial relationships.
Precious Idogun indicated no relevant financial relationships.
Nathan Seepaulsing indicated no relevant financial relationships.
Richard Brown indicated no relevant financial relationships.
Coppola Domenico indicated no relevant financial relationships.
Scott Corbett indicated no relevant financial relationships.
Mindy Ward, DO1, Precious Idogun, MD2, Nathan Seepaulsing, 2, Richard Brown, MD2, Coppola Domenico, MD3, Scott Corbett, MD2. P1279 - Langerhans Cell Histiocytosis Mimicking Inflammatory Bowel Disease: A Rare Presentation of Multiorgan Myeloid Neoplasm in a Geriatric Patient, ACG 2021 Annual Scientific Meeting Abstracts. Las Vegas, Nevada: American College of Gastroenterology.