Progresivna multifokalna levkoencefalopatija po rituksimabu pri bolniku z recidivnim limfomom plaščnih celic v popolni remisiji Progressive multifocal leukoencephalopathy following rituximab therapy in a patient with a relapsed mantle cell lymphoma in complete remission

Abstract

Progresivna multifokalna levkoencefalopatija (PML) je redko in običajno smrtno demielinizacijsko obolenje možganov, ki se razvije skoraj izključno pri bolnikih z imunsko pomanjkljivostjo in je posledica reaktivacije latentne okužbe s polioma virusom JC. V zadnjih letih so opisani tudi primeri PML po zdravljenju z rituksimabom. Predstavljamo naš prvi potrjeni primer PML pri 55-letnem HIV-negativnem bolniku z recidivnim limfomom plaščnih celic, pri katerem smo raka uspešno zdravili s kemoterapijo in rituksimabom ter z avtologno presaditvijo perifernih krvotvornih matičnih celic (PKMC). S primerom želimo opozoriti na pomembnost PML v diferencialni diagnozi pri bolnikih z napredujočo nevrološko simptomatiko in imunsko pomanjkljivostjo po zdravljenju limfoma z rituksimabom ter pomembnost zgodnjega prepoznavanja te bolezni z dokazom JC-virusne okužbe v likvorju ali v vzorcu stereotaktične biopsije možganov.

Abstract (Eng)

Progressive multifocal leukoencephalopathy (PML) is a rare and usually fatal demyelinating disease of the brain that is developed almost exclusively in patients with immunodeficiency and is a result of a latent infection with the JC polyomavirus. In recent years, several cases of PML have been reported following rituximab therapy. We present our first confirmed case of PML in a 55-year-old HIV-negative patient with a relapsed mantle cell lymphoma who was treated successfully with chemotherapy, rituximab and autologous transplantation of peripheral haematopoietic stem cells (PHSC). By reporting this case we wish to draw attention to the importance of PML in differential diagnosis of patients with a progressing neurological symptoms and immunodeficiency following lymphoma treatment with rituximab and stress the importance of early detection of this disease with evidence of JC virus infection in the cerebrospinal fluid or stereotactic brain biopsy sample.