A rare type of non-Hodgkin lymphoma and is characterized by a malignant proliferation

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 West Nile Virus varies in presentation from asymptomatic to a febrile illness often associated with malaise, weakness and maculopapular rash. West Nile neuro-invasive disease often manifests as meningitis, encephalitis, and less commonly acute flaccid paralysis in a "polio-like" presentation. Acute transverse myelitis (ATM) is a rare manifestation. We present a case of neuro-invasive West Nile Virus infection with radiographic evidence of longitudinally extensive transverse myelitis (LETM), a subset of ATM.The possible role of JC virus in determining urinary tract involvement has only recently been recognized. The case of a man with laboratory-confirmed JC virus replication in the urine after a maintenance schedule of rituximab administered for a lymphoproliferative disorder is reported herein. The patient developed severe renal and urinary tract impairment, characterized by the onset of nephropathy, bilateral ureteral strictures, and a serious reduction in vesical compliance, ultimately requiring an ileal neobladder configuration. The renal and urinary tract involvement was finally attributed to JC virus reactivation. This observation suggests that renal and urinary tract diseases related to JC virus might be associated with long-term rituximab treatment.

Intravascular lymphoma is a rare type of non-Hodgkin lymphoma and is characterized by a malignant proliferation of lymphocytes inside the lumen of blood vessels. Intravascular lymphoma is primarily an extranodal lymphoma, with skin and central nervous system reported as the most affected sites. Most cases (approximately 90%) consist of B-cell origin, but T and natural killer (NK) cell lineages have also been reported. Intravascular NK/T-cell lymphoma is typically related to the Epstein-Barr virus (EBV), expression of cytotoxic markers, and an aggressive course with an adverse prognosis. Cutaneous intravascular NK/T-cell lymphoma can be difficult to differentiate, both clinically and immunohistologically, from other cutaneous lymphomas, such as intravascular cutaneous anaplastic large cell lymphoma, extranodal NK/T-cell lymphoma (nasal type), or blastic plasmacytoid dendritic cell neoplasm. Within the cutaneous intravascular NK/T-cell lymphomas, most reported cases are of NK cell phenotype with a constant relation to EBV, and most patients die within 6 months. Only sporadic cases with a (NK-like) T-cell phenotype are described, with a variable immunophenotype and disease course. We describe the first case, to our knowledge, of a cutaneous EBV intravascular cytotoxic CD4+ T-cell lymphoma, with a seemingly indolent disease course.

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Sofia
Journal Co-ordinator
Journal of Rare Disorders: Diagnosis & Therapy