title: Localized pulmonary crystal-storing histiocytosis complicating pulmonary mucosa-associated lymphoid tissue lymphoma presenting with multiple mass lesions.
abstract: Crystal-storing histiocytosis (CSH) is an uncommon finding in lymphoplasmacytic disorders that presents histiocytes with abnormal intralysosomal accumulations of immunoglobulin light chains as crystals of unknown etiology. A 38-year-old woman with antiphospholipid syndrome had a surgical lung biopsy because of multiple lung mass lesions. In a right middle lobe lesion, lymphoplasmacytic cells had a monocytoid appearance, destructive lymphoepithelial lesions, and positive immunoglobulin heavy chain (IGH) gene rearrangements. A right upper lobe lesion manifested proliferating rounded histiocytes with abundant, deeply eosinophilic cytoplasm and negative IGH gene rearrangements. Electron microscopy and mass spectrometry revealed a case of pulmonary CSH: abnormal proliferation of the immunoglobulin κ chain of a variable region that may be crystallized within plasma cells and histiocytes. We report a rare case of localized pulmonary CSH complicating pulmonary mucosa-associated lymphoid tissue lymphoma with multiple mass lesions. We demonstrate advances in the understanding of the pathogenesis of CSH by various analyses of these lesions.
authors.affiliation: Department of Analytic Human Pathology, Graduate School of Medicine, Nippon Medical School, Tokyo 113-0031, Japan; Department of Pulmonary Medicine and Oncology, Graduate School of Medicine, Nippon Medical School, Tokyo 113-8602, Japan.
authors.forename: Nariaki
authors.initials: N
authors.lastname: Kokuho
authors.affiliation: Department of Analytic Human Pathology, Graduate School of Medicine, Nippon Medical School, Tokyo 113-0031, Japan. Electronic address: terasaki@nms.ac.jp.
authors.forename: Yasuhiro
authors.initials: Y
authors.lastname: Terasaki
authors.affiliation: Department of Analytic Human Pathology, Graduate School of Medicine, Nippon Medical School, Tokyo 113-0031, Japan.
authors.forename: Shinobu
authors.initials: S
authors.lastname: Kunugi
authors.affiliation: Department of Pulmonary Medicine and Oncology, Graduate School of Medicine, Nippon Medical School, Tokyo 113-8602, Japan; Nippon Medical School, Chiba Hokusoh Hospital, Respiratory Disease Center, Chiba 270-1694, Japan.
authors.forename: Naomi
authors.initials: N
authors.lastname: Onda
authors.affiliation: Department of Analytic Human Pathology, Graduate School of Medicine, Nippon Medical School, Tokyo 113-0031, Japan.
authors.forename: Hirokazu
authors.initials: H
authors.lastname: Urushiyama
authors.affiliation: Department of Analytic Human Pathology, Graduate School of Medicine, Nippon Medical School, Tokyo 113-0031, Japan.
authors.forename: Mika
authors.initials: M
authors.lastname: Terasaki
authors.affiliation: Nippon Medical School, Chiba Hokusoh Hospital, Respiratory Disease Center, Chiba 270-1694, Japan.
authors.forename: Mitsunori
authors.initials: M
authors.lastname: Hino
authors.affiliation: Department of Pulmonary Medicine and Oncology, Graduate School of Medicine, Nippon Medical School, Tokyo 113-8602, Japan.
authors.forename: Akihiko
authors.initials: A
authors.lastname: Gemma
authors.affiliation: Department of Pathology, Nippon Medical School, Chiba Hokusoh Hospital, Chiba 270-1694, Japan.
authors.forename: Tsutomu
authors.initials: T
authors.lastname: Hatori
authors.affiliation: Department of Analytic Human Pathology, Graduate School of Medicine, Nippon Medical School, Tokyo 113-0031, Japan.
authors.forename: Akira
authors.initials: A
authors.lastname: Shimizu
mesh_terms: D000328:Adult; D014408:Biomarkers, Tumor / Q000032:analysis* / Q000235:genetics; D002853:Chromatography, Liquid; D003460:Crystallization; D005260:Female; D015321:Gene Rearrangement; D050438:Genes, Immunoglobulin Heavy Chain; D006644:Histiocytes / Q000276:immunology* / Q000648:ultrastructure; D015614:Histiocytosis / Q000235:genetics / Q000276:immunology* / Q000473:pathology; D006801:Humans; D007145:Immunoglobulin kappa-Chains / Q000032:analysis*; D007150:Immunohistochemistry; D008175:Lung Neoplasms / Q000235:genetics / Q000276:immunology* / Q000473:pathology / Q000648:ultrastructure; D018442:Lymphoma, B-Cell, Marginal Zone / Q000235:genetics / Q000276:immunology* / Q000648:ultrastructure; D008854:Microscopy, Electron; D016133:Polymerase Chain Reaction; D049268:Positron-Emission Tomography; D053719:Tandem Mass Spectrometry; D014057:Tomography, X-Ray Computed
chemical_list: D014408:Biomarkers, Tumor; D007145:Immunoglobulin kappa-Chains
keywords: Crystal-storing histiocytosis; Liquid chromatography–tandem mass spectrometry; MALT; Mucosa-associated lymphoid tissue lymphoma; Pulmonary mucosa-associated lymphoid tissue lymphoma