Crystal-storing histiocytosis associated with only one of two consecutive, but genetically unrelated B-cell lymphomas.
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.
László
Renáta
R
Department of Pathology, Faculty of Medicine, University of Pécs, 12 Szigeti Street, H-7624, Pécs, Hungary.
Degrell
Péter
P
Kellermayer
Miklós
M
Bollmann
Daniel
D
Egyed
Miklós
M
Seress
László
L
Seres
László
L
Pajor
László
L
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
D007143:Immunoglobulin Heavy Chains; D007147:Immunoglobulin Light Chains