title: Nonnecrobiotic necrobiotic xanthogranuloma as an initial manifestation of paraproteinemia and small lymphocytic lymphoma in a patient with Sjögren syndrome.
abstract: We report a unique case of periocular nonnecrobiotic necrobiotic xanthogranuloma in a 52-year-old white woman with Sjögren syndrome who was subsequently found to have an immunoglobulin G paraproteinemia and coexisting small lymphocytic lymphoma. Therapy with fludarabine, cytoxan, and rituximab (FCR) resulted in a dramatic resolution of her sicca symptoms and periocular xanthogranulomas. This case further illustrates the association of hematolymphoid disorders with cutaneous xanthogranulomatous disease and the importance of additional appropriate laboratory and radiologic investigation for the accurate diagnosis of an underlying malignancy.
authors: Department of Hematopathology, H. Lee Moffitt Cancer Center, University of South Florida, USA. mojdeh.naghashpour@moffitt.org
authors: Mojdeh
authors: M
authors: Naghashpour
authors: Reza
authors: R
authors: Setoodeh
authors: Lynn
authors: L
authors: Moscinski
authors: Gregoire
authors: G
authors: Bergier
authors: Timothy
authors: T
authors: McCardle
authors: Frank
authors: F
authors: Glass
authors: Lubomir
authors: L
authors: Sokol
mesh_terms: D058846:Antibodies, Monoclonal, Murine-Derived / Q000627:therapeutic use; D000970:Antineoplastic Agents / Q000627:therapeutic use; D001706:Biopsy; D003520:Cyclophosphamide / Q000627:therapeutic use; D004359:Drug Therapy, Combination; D005143:Eyelids / Q000473:pathology; D005260:Female; D006801:Humans; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D015451:Leukemia, Lymphocytic, Chronic, B-Cell / Q000150:complications* / Q000276:immunology / Q000473:pathology; D008875:Middle Aged; D058252:Necrobiotic Xanthogranuloma / Q000188:drug therapy / Q000209:etiology* / Q000276:immunology / Q000473:pathology; D010265:Paraproteinemias / Q000150:complications* / Q000188:drug therapy / Q000276:immunology / Q000473:pathology; D000069283:Rituximab; D012859:Sjogren's Syndrome / Q000150:complications* / Q000188:drug therapy / Q000276:immunology / Q000473:pathology; D016896:Treatment Outcome; D014740:Vidarabine / Q000031:analogs & derivatives / Q000627:therapeutic use
chemical_list: D058846:Antibodies, Monoclonal, Murine-Derived; D000970:Antineoplastic Agents; D007166:Immunosuppressive Agents; D000069283:Rituximab; D003520:Cyclophosphamide; D014740:Vidarabine; C024352:fludarabine