title: Crystal-storing histiocytosis associated with only one of two consecutive, but genetically unrelated B-cell lymphomas.
abstract: A localized crystal-storing histiocytosis (CSH) making the underlying marginal zone lymphoma (MZL) hardly discernible microscopically is described. Image analysis of the hyper electron dense crystals localized light microscopically in swollen histiocytic cells exhibited a major equatorial periodicity of 6.6 nm. Rarely, crystals of this type were detected within plasma cells, but were always surrounded by smooth membrane in contrast to Russell bodies. IgM/lambda restriction and VH3-21*02, DH4-17*01, JH4*02 gene usage were detected behind the lesion. Within 26 months, a genetically unrelated lymphoma of CD5-CD20-CD23-positive phenotype with a different VH1-24*01, DH2-21*02, JH2*01 heavy chain rearrangement, but with the same light chain gene usage, was identified without CSH. This might indicate that the unique condition responsible for the crystal formation is likely to rely on the sequence of the first clonally rearranged heavy chain exhibiting much higher CDRIII pI value (6.0) than the average.
authors.affiliation: Department of Pathology, Faculty of Medicine, University of Pécs, 12 Szigeti Street, H-7624, Pécs, Hungary.
authors.forename: Renáta
authors.initials: R
authors.lastname: László
authors.forename: Péter
authors.initials: P
authors.lastname: Degrell
authors.forename: Miklós
authors.initials: M
authors.lastname: Kellermayer
authors.forename: Daniel
authors.initials: D
authors.lastname: Bollmann
authors.forename: Miklós
authors.initials: M
authors.lastname: Egyed
authors.forename: László
authors.initials: L
authors.lastname: Seress
authors.forename: László
authors.initials: L
authors.lastname: Seres
authors.forename: László
authors.initials: L
authors.lastname: Pajor
mesh_terms: D000368:Aged; D003460:Crystallization; D005260:Female; D005434:Flow Cytometry; D015326:Gene Rearrangement, B-Lymphocyte, Heavy Chain; D015328:Gene Rearrangement, B-Lymphocyte, Light Chain; D006644:Histiocytes / Q000473:pathology*; D015614:Histiocytosis / Q000473:pathology*; D006801:Humans; D007143:Immunoglobulin Heavy Chains / Q000235:genetics; D007147:Immunoglobulin Light Chains / Q000235:genetics; D002479:Inclusion Bodies / Q000473:pathology*; D008198:Lymph Nodes / Q000473:pathology; D016393:Lymphoma, B-Cell / Q000235:genetics / Q000473:pathology*; D009378:Neoplasms, Multiple Primary / Q000235:genetics / Q000473:pathology*; D016133:Polymerase Chain Reaction
chemical_list: D007143:Immunoglobulin Heavy Chains; D007147:Immunoglobulin Light Chains