Localized pulmonary crystal-storing histiocytosis complicating pulmonary mucosa-associated lymphoid tissue lymphoma presenting with multiple mass lesions

• Published in: Human pathology 2017-07, Vol.65, p.180-186
• Main Authors: Kokuho, Nariaki, MD, PhD, Terasaki, Yasuhiro, MD, PhD, Kunugi, Shinobu, MD, PhD, Onda, Naomi, MD, Urushiyama, Hirokazu, MD, PhD, Terasaki, Mika, MD, PhD, Hino, Mitsunori, MD, PhD, Gemma, Akihiko, MD, PhD, Hatori, Tsutomu, MD, PhD, Shimizu, Akira, MD, PhD
• Format: Article
• Language: English
• Subjects: Adult; Biomarkers, Tumor - analysis; Biomarkers, Tumor - genetics; Biopsy; Bone marrow; Chromatography, Liquid; Cloning; Crystal-storing histiocytosis; Crystallization; Crystals; Cytoplasm; Disease; Female; Gene Rearrangement; Genes, Immunoglobulin Heavy Chain; Histiocytes - immunology; Histiocytes - ultrastructure; Histiocytosis - genetics; Histiocytosis - immunology; Histiocytosis - pathology; Histology; Histopathology; Humans; Immunoglobulin kappa-Chains - analysis; Immunoglobulins; Immunohistochemistry; Lasers; Liquid chromatography–tandem mass spectrometry; Lung Neoplasms - genetics; Lung Neoplasms - immunology; Lung Neoplasms - pathology; Lung Neoplasms - ultrastructure; Lungs; Lymphoma; Lymphoma, B-Cell, Marginal Zone - genetics; Lymphoma, B-Cell, Marginal Zone - immunology; Lymphoma, B-Cell, Marginal Zone - ultrastructure; MALT; Mass spectrometry; Medical screening; Microscopy; Microscopy, Electron; Mucosa-associated lymphoid tissue lymphoma; Multiple myeloma; Pathogenesis; Pathology; Peptides; Polymerase Chain Reaction; Positron-Emission Tomography; Proteins; Pulmonary mucosa-associated lymphoid tissue lymphoma; Scientific imaging; Tandem Mass Spectrometry; Tomography; Tomography, X-Ray Computed
• ISSN: 0046-8177; 1532-8392
• DOI: 10.1016/j.humpath.2016.10.028

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.