A CASE OF ERDHEIM-CHESTER DISEASE IDENTIFIED SIX MONTHS AFTER LUNG TRANSPLANT

Abstract

TYPE: Case Report TOPIC: Transplantation INTRODUCTION: Erdheim-Chester disease is a rare type of non-Langerhans histiocytosis that can manifest with bone pain, marked elevation of alkaline phosphatase, and osteosclerotic lesions of long bones. CASE PRESENTATION: A 37-year-old female, bilateral lung transplant recipient (10/2020) with a history of post-COVID-19 acute respiratory distress syndrome, complicated by acute cellular rejection, antibody-mediated rejection, and refractory gastroparesis, was found to have Erdheim-Chester disease in the work-up of infiltrative pattern of liver enzymes at 6-months after transplant. DISCUSSION: The early postoperative course was complicated as described above; during her most recent hospital admission, she had an isolated elevation of alkaline phosphatase (208 U/L) with no obvious gastrointestinal symptoms, excluding gastroesophageal reflux or other hepatobiliary pathology. Over the course of one week, her alkaline phosphatase increased to 928 U/L, and the patient described a new onset, mild and non-specific lower tibial pain. A gamma-glutamyl transferase was elevated, necessitating a skeletal work-up. A bone scan and lower extremity radiographs both showed subtle, patchy, intramedullary sclerosis and cortical thickening in the right lower extremity. Together, her bone pain, elevated alkaline phosphatase, and radiologic features were consistent with Erdheim-Chester disease. Although a bone biopsy was warranted, given her guarded prognosis, this intervention was not pursued. CONCLUSIONS: Erdheim-Chester disease generally requires careful observation for disease progression. Unfortunately, in a lung transplant recipient with worsening allograft function and repeated hospitalization, the prognosis is poor. DISCLOSURE: Nothing to declare. KEYWORD: Erdheim-Chester Disease