Langerhans cell histiocytosis mimicking trauma in a 2-year-old patient with T-cell acute lymphoblastic leukemia — a case report
Langerhans cell histiocytosis (LCH) is a rare clonal disorder of myeloid dendritic cells that can affect bones and multiple organs.
Langerhans cell histiocytosis (LCH) is a rare clonal disorder of myeloid dendritic cells that can affect bones and multiple organs. Clinical manifestations vary depending on organ involvement. Activating mutations in the MAPK pathway, particularly BRAF V600E, are present in a significant proportion of cases. The coexistence of LCH and acute lymphoblastic leukemia (ALL) is extremely rare and poses diagnostic and therapeutic challenges. We report the case of a 2-year-old boy treated for T-cell acute lymphoblastic leukemia diagnosed in September 2021 at a tertiary pediatric hematooncology and transplant center in Poland, who developed a rapidly progressive osteolytic lesion of the distal humerus after minor trauma. The initial radiograph was normal. Two weeks later, repeat imaging showed cortical thinning, a permeative lytic pattern, and periosteal reaction. B-mode ultrasound and color Doppler demonstrated a hypervascular solid mass rather than fluid collections. Biopsy confirmed LCH. Further imaging revealed multifocal skeletal involvement. Treatment required careful modification of the ongoing leukemia protocol to reduce cumulative toxicity, while systemic vinblastine and prednisone therapy was initiated for LCH. Because of the aggressive and multisystem disease course, the patient subsequently underwent allogeneic hematopoietic stem cell transplantation (HSCT). Following HSCT, the patient achieved complete hematologic remission of T-ALL, with negative minimal residual disease (MRD) and 100% donor chimerism. PET-CT showed a gradual reduction in metabolic activity of LCH lesions. This case underscores the importance of early biopsy, multimodal imaging, and coordinated multidisciplinary management in immunocompromised children presenting with rapidly evolving bone lesions.
Bizonyítékszint: Számítógépes vagy elméleti. Modellből vagy adatbányászatból származó jel.
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