An overview on bone manifestations in Gaucher disease.

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Abstract

[Indexed for MEDLINE] 20. R I Med J (2013). 2022 Aug 1;105(6):41-45. Describing IgA Myeloma: An Immunophenotypic and Molecular Approach. Akgun Y(1), Baykara Y(2), Hacking SM(3), Langlie J(4), Huberman MA(4), Espejo AP(5), Chapman J(1), Poveda J(1). Author information: (1)Department of Pathology and Laboratory Medicine, University of Miami and Jackson Health System, Miami, Florida. (2)Department of Pathology and Laboratory Medicine, Rhode Island Hospital and Lifespan Medical Center, The Warren Alpert Medical School of Brown University, Providence, Rhode Island. (3)Department of Pathology and Laboratory Medicine, The Warren Alpert Medical School of Brown University; Department of Pathology and Laboratory Medicine, Donald and Barbara Zucker School of Medicine at Northwell. (4)University of Miami Miller School of Medicine, University of Miami and Jackson Health System, Miami, Florida. (5)Department of Hematology and Medical Oncology, University of Miami, Jackson Health System, Miami, Florida. Plasma cell myeloma (PCM) is defined as a clonal disease of terminally differentiated plasma cells that secrete immunoglobulin. The biologic underpinnings of IgA-type multiple myeloma's (IgAMM) aggressive nature, including its increased morbidity and mortality, have not been elucidated. We describe the clinical, phenotypic, and cytogenetic characteristics of IgA-MM. Flow-cytometry analysis was performed to phenotype clonal plasma cell populations, and interface with fluorescent in situ hybridization (iFISH) to exploit cytogenetics to determine risk stratification; 68.1% of cases were of intermediate or high risk. On flow cytometry, samples from our IgA-PCM cohort revealed less frequent CD56 expression when compared to samples with other PCM subtypes. Our study demonstrated lower frequency of CD56 expression (52.8%). We hypothesize that loss of CD56 may play a significant role in the aggressive behavior of IgA-PCM due to the loss of cell-to-cell adhesion resulting in a higher propensity for extramedullary presentation.