Progresívna multifokálna leukoencefalopatia (PML) bola prvýkrát popísaná v roku 1958 u pacienta s lymfoproliferatívnym ochorením. V roku 1971 bol izolovaný John Cunninghamov vírus (JCV), ktorý je zodpovedný za vznik tohto ochorenia. V našej práci popisujeme raritný prípad 62-ročného pacienta s chronickou lymfocytovou leukémiou a PML. Zároveň diskutujeme o diagnostických a terapeutických výzvach a ponúkame návrhy prevencie rozvoja PML. Hlavnými závermi práce sú: 1. Potrebný je pravidelný monitoring sérových hladín imunoglobulínov a pomeru CD4+ : CD8+ T-lymfocytov, podanie intravenóznych imunoglobulínov je potrebné zvážiť pri ich poklese. 2. Pri poklese pomeru CD4+ : CD8+ T-lymfocytov a nemožnosti podať intravenózne imunoglobulíny musíme zvážiť pokračujúci prínos podávania imunoterapie. 3. Pri objavení sa neurologickej symptomatológie u pacienta, ktorý podstupuje imunosupresívnu liečbu, musí lekár vysloviť podozrenie na možný rozvoj PML.

The definition ”Progressive Multifocal Leukoencephalopathy” (PML) was first used in 1958 to describe a fatal demyelinating central nervous system (CNS) disease in patients with lymphoproliferative disorders. In 1971, the virus responsible for the disease was isolated and named John Cunningham virus (JCV). We present a rare case of a 62-year-old male with chronic lymphocytic leukemia and PML. In our work, we discuss the diagnostic and therapeutic challenges and offer suggestions for preventing PML development. The main learning points are: 1. Regularly check the level of immunoglobulins and the CD4+ : CD8+ T-cell ratio, intravenous administration of immunoglobulins should be considered when recording their reduction. 2. In checking the CD4+ : CD8+ T-cell ratio and verifying the impossibility of raising the level of immunoglobulins, we must weigh the possible benefits of continuing treatment with monoclonal antibody against the risks. 3. Physicians should maintain a high index of suspicion for the development of PML in patients under treatment with monoclonal antibodies, especially when there is a new development of neurological signs or symptoms.

Department of Haematology and Transfusion Medicine National Centre of Haemostasis and Thrombosis Jessenius Faculty of Medicine in Martin Comenius University in Bratislava Slovakia

Department of Neurology Jessenius Faculty of Medicine in Martin Comenius University in Bratislava Slovakia

Department of Pathological Anatomy Jessenius Faculty of Medicine in Martin Comenius University in Bratislava Slovakia

Progresívna multifokálna leukoencefalopatia spojená s rituximabom