title: A case of intravascular large B cell lymphoma: New clinical and immunohistochemical findings.
abstract: Intravascular large B cell lymphoma (IVLBCL) is a rare extranodal non-Hodgkin lymphoma characterized by proliferation of malignant cells within the lumen of small vessels, with a predilection for the CNS and the skin. IVLBCL clinical course is highly aggressive, clinical signs and symptoms are not specific and may consist of neurological and cognitive impairment, fever of unknown origin and cutaneous lesions, lacking of a typical neuroimaging pattern. For all these reasons the diagnosis is commonly missed and the exitus is frequent, therefore post mortem evaluation is necessary to clarify the clinical history. We present a case of IVLBCL in a 62-year-old woman with unusual symptomatology, mimicking a vascular, multi-infarctual cerebropathy. Hachinski Ischemic Score was 7 suggesting a vascular dementia. Autopsy was unable to define the nature of the disease. Immunohistochemical analysis for cluster of differentiation 20 (CD20) revealed the ubiquitous presence of malignant lymphoid B-cells into the vessel of all organs analyzed, allowing the definitive diagnosis of IVLBCL. The atypical cells expressed high levels of anti-apoptotic proteins B-cell lymphoma 2 (Bcl-2) and Galectin-3, and showed cellular myelocytomatosis (c-Myc) staining in <50% of tumor nuclei. Conversely, cells were immunonegative for multiple myeloma-1 (MUM1), CD3, CD44, CD30, CD34 and CD133. Fluorescent in situ hybridization analysis for MYC rearrangements was negative. The high expression of Galectin-3 provides new insights in the understanding of molecular pathogenesis of IVLBCL; indeed, such a finding represents a prognostic factor for other types of lymphoma and should, in the same way, be taken into account in IVLBCL.
authors.affiliation: Advanced Biomedical Sciences, Pathology Section, University of Naples "Federico II", Italy.
authors.forename: Gelsomina
authors.initials: G
authors.lastname: Mansueto
authors.affiliation: Department of Clinical and Experimental Medicine, University "Magna Graecia" of Catanzaro, Italy.
authors.forename: Anna
authors.initials: A
authors.lastname: Di Vito
authors.affiliation: Department of Anesthesiologic, Surgical and Emergency Science, Second University of Naples, Italy.
authors.forename: Clara
authors.initials: C
authors.lastname: Belluomo
authors.affiliation: Department of Anesthesiologic, Surgical and Emergency Science, Second University of Naples, Italy.
authors.forename: Patrizia
authors.initials: P
authors.lastname: Murino
authors.affiliation: Advanced Biomedical Sciences, Pathology Section, University of Naples "Federico II", Italy.
authors.forename: Valentina
authors.initials: V
authors.lastname: Natella
authors.affiliation: Department of Health Science, University "Magna Graecia" of Catanzaro, Italy.
authors.forename: Caterina
authors.initials: C
authors.lastname: Camastra
authors.affiliation: Department of Health Science, University "Magna Graecia" of Catanzaro, Italy.
authors.forename: Ivan
authors.initials: I
authors.lastname: Presta
authors.affiliation: Department of Health Science, University "Magna Graecia" of Catanzaro, Italy.
authors.forename: Natalia
authors.initials: N
authors.lastname: Malara
authors.affiliation: Advanced Biomedical Sciences, Pathology Section, University of Naples "Federico II", Italy.
authors.forename: Gaetano
authors.initials: G
authors.lastname: de Rosa
authors.affiliation: Department of Health Science, University "Magna Graecia" of Catanzaro, Italy.
authors.forename: Giuseppe
authors.initials: G
authors.lastname: Donato
authors.affiliation: Department of Health Science, University "Magna Graecia" of Catanzaro, Italy.
authors.forename: Chiara
authors.initials: C
authors.lastname: Mignogna
mesh_terms: D018951:Antigens, CD20 / Q000378:metabolism; D001921:Brain / Q000098:blood supply / Q000378:metabolism* / Q000473:pathology*; D005260:Female; D037502:Galectin 3 / Q000378:metabolism; D006801:Humans; D007150:Immunohistochemistry; D016393:Lymphoma, B-Cell / Q000175:diagnosis / Q000378:metabolism* / Q000473:pathology*; D008875:Middle Aged; D019253:Proto-Oncogene Proteins c-bcl-2 / Q000378:metabolism; D016271:Proto-Oncogene Proteins c-myc
chemical_list: D018951:Antigens, CD20; D037502:Galectin 3; D019253:Proto-Oncogene Proteins c-bcl-2; D016271:Proto-Oncogene Proteins c-myc
keywords: Galectin 3; Hachinski; IVLBCL; Immunohistochemistry; vascular dementia