Crystal-storing histiocytosis and Bing-Neel syndrome are two diseases induced by paraproteins. Herein, we report a rare case of crystal-storing histiocytosis associated with Bing-Neel-like neurological manifestations in the context of a small B-cell lymphoma with plasmacytic differentiation, presumed to be a marginal zone lymphoma.

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http://www.ncbi.nlm.nih.gov/pmc/articles/PMC8650757PMC
http://dx.doi.org/10.1002/ccr3.5202DOI Listing

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  • Crystal-storing histiocytosis (CSH) is a rare condition where abnormal protein crystals accumulate in the body, and when it affects the CNS, it can cause persistent imaging abnormalities.* -
  • The study describes one local case of CNS CSH alongside 10 other cases, noting that 9 had brain parenchyma involvement and some cases were linked to conditions like lymphoma.* -
  • While CNS CSH is uncommon, comprehensive evaluation for related malignancies is crucial, and treatment focuses on addressing these underlying conditions.*
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  • * The case study illustrates a patient with kappa chain myeloma who exhibited Gaucher-like crystal-storing histiocytosis in bone marrow, identified through various methods including biopsy and next generation sequencing (NGS).
  • * Findings indicated the presence of distinct rhomboid crystals and several genetic mutations linked to poorer survival rates in myeloma patients, suggesting that pathologists should consider crystal-storing histiocytosis when identifying such crystals in bone marrow.
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