Abstract

BackgroundLangerhans cell tumors are rare clonal disorders characterized by neoplastic proliferation of dendritic cells that can be further classified into the subtypes Langerhans cell histiocytosis and Langerhans cell sarcoma, which are rare neoplasms exhibiting aggressive features and a poor prognosis. In addition to illustrating the refractoriness and poor outcomes of multisystem Langerhans cell histiocytosis in adults, specific events in this case highlight important characteristics of disease biology that warrant detailed discussion and exposition to a wider audience.Case presentationWe describe the case of a 42-year-old Caucasian man with Langerhans cell histiocytosis diagnosed from a lesion on the left arm that presented with constitutional symptoms, early satiety, and weight loss. Esophagogastroduodenoscopy showed extensive esophageal and duodenal involvement by Langerhans cell histiocytosis with features of Langerhans cell sarcoma. He was initially treated for Langerhans cell histiocytosis with low doses of cytarabine until he eventually presented clear transformation to acute monoblastic leukemia with complex karyotype that could not be properly controlled, leading eventually to death.ConclusionsLangerhans cell histiocytosis remains an exceedingly rare entity in adults, frequently presenting as multisystem disease with risk organ involvement. Langerhans cell sarcoma represents an aggressive subtype with extremely poor prognosis for which intensive acute myeloid leukemia induction should be strongly considered.

Highlights

  • Langerhans cell tumors are rare clonal disorders characterized by neoplastic proliferation of dendritic cells that can be further classified into the subtypes Langerhans cell histiocytosis and Langerhans cell sarcoma, which are rare neoplasms exhibiting aggressive features and a poor prognosis

  • Langerhans cell tumors are rare clonal disorders characterized by neoplastic proliferation of dendritic cells with distinctive protein expression (CD1a/langerin/S100) and ultrastructural features (Birbeck granules) with Langerhans cell histiocytosis (LCH) and Langerhans cell sarcoma (LCS) subtypes [1,2,3]

  • We report a case of a patient with a presentation with symptomatic multisystem multifocal Langerhans Cell Histiocytosis (LCH) disease with early suggestion of leukemic transformation with particular sensitivity to growth factors

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Summary

Introduction

Langerhans cell tumors are rare clonal disorders characterized by neoplastic proliferation of dendritic cells that can be further classified into the subtypes Langerhans cell histiocytosis and Langerhans cell sarcoma, which are rare neoplasms exhibiting aggressive features and a poor prognosis. Case presentation A 42-year-old Caucasian man presented to our hospital with acute onset of fatigue, nausea, vomiting, early satiety, diarrhea, and weight loss 3 months after LCH had been diagnosed on the basis of biopsy of an asymptomatic lesion on the left arm. His physical examination was pertinent for tender hepatosplenomegaly and multiple eczematous papular lesions on the trunk and extremities. His bone marrow biopsy (BMBx) showed no LCH or acute myeloid leukemia (AML) He was started on intravenous cytarabine 100 mg/m2 daily for 5 days every 4 weeks, which led to improvement but no resolution of his skin lesions or gastrointestinal (GI) symptoms. This adjustment led to clinical improvement, but his LDH level continued to rise, leading to repeat BMBx performed after cycle 8, which showed no evidence of AML

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