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Case Reports

Abstract

Xanthogranuloma predominantly presents in pediatric patients as a solitary lesion. Multiple xanthogranulomas in adults are unusual and rarely associated with hematologic malignancies. Here, we describe a case of multiple adult xanthogranuloma (AXG) in a patient who was subsequently diagnosed with chronic myelomonocytic leukemia (CMML). A 55-year-old female with a past medical history of alcohol use disorder, mosaic Turner syndrome, anxiety, and depression presented to an outside dermatologist in 2017 for skin lesions of unknown duration. Physical examination revealed yellow to red flat-topped papules and nodules scattered on the trunk, upper arms, and face, measuring up to 10 mm. Multiple biopsies were performed, which demonstrated diffuse histiocytic infiltrate with focal giant cells, foam cells, lymphocytes, and rare eosinophils consistent with AXG. A complete blood count showed normal findings except mild monocytosis (16%, 1150/μL). A computed tomography scan was notable for hepatomegaly, and a subsequent liver biopsy demonstrated steatohepatitis. She was admitted to our hospital in 2022 for hypoxic respiratory failure and pericarditis secondary to coxsackievirus infection. During admission, she was found to be pancytopenic with monocytosis (50%, 1980/μL). Next-generation sequencing of the bone marrow biopsy was positive for a pathogenic KRAS mutation, and she was ultimately diagnosed with CMML. She underwent chemotherapy and bone marrow transplant. One year post-transplant, her AXG lesions improved dramatically. We present this case to highlight the association between AXG and hematologic malignancies and stress the importance of hematologic workup in patients with multiple AXG.