PROGRESSIVE MULTIFOCAL LEUKOENCEPHALOPATHY AFTER HEMATOPOIETIC STEM CELL TRANSPLANTATION: A CASE REPORT

PROGRESSIVE MULTIFOCAL LEUKOENCEPHALOPATHY AFTER HEMATOPOIETIC STEM CELL TRANSPLANTATION: A CASE REPORT

Evento: SPPC 2021

Poster Número: 045

Autores e Afiliações:

Daniela Fonseca e Silva1*, Joana Marinho-Dias1*, Ana Torgal1, Delfim Duarte2, Cátia Sol2, Rui Medeiros1, Augusto Ferreira3, Carlos Pinho-Vaz4, António Campos-Júnior4, Inês Baldaque1.

*Both authors contributed equally to this work

1 – Virology Service, Portuguese Oncology Institute of Porto FG, E.P.E.

2 – Department of Onco-Hematology – Portuguese Oncology Institute of Porto FG, E.P.E.

3 – Neurology Service, Portuguese Oncology Institute of Porto FG, E.P.E.

4 – Bone Marrow Transplant Unit – Portuguese Oncology Institute of Porto FG, E.P.E.

Progressive multifocal leukoencephalopathy (PML) is a rare neurodegenerative disease of the central nervous system (CNS), which affects white brain matter and is caused by reactivation of the JC polyomavirus (JCV). There is no specific therapy for JCV infection or PML, and the main approach involves reversing immunosuppression. We present the case of a 22year-old male with a diagnosis of relapsed Hodgkin’s lymphoma, referenced to our Institution in October 2020 for allogeneic hematopoietic stem cell transplant (HSCT). He underwent HSCT from a non-related donor, after reduced intensity conditioning with fludarabine, busulfan and anti-thymocyte globulin. He received tacrolimus and mycophenolate mofetil (MMF) as graft versus host disease (GVHD) prophylaxis. The patient developed cutaneous and ocular acute GVHD and was currently under immunosuppression with prednisolone 20mg/day and MMF 1000 mg bid. In October 2021, the patient suffered a car accident due to diminished reflexes. Upon evaluation he reported sleepiness since the previous 2 weeks and labial commissure deviation in the last four days. Neurological evaluation revealed discrete dysarthria, tongue protrusion and face hemiparesis but no meningeal signs. CT scan described an area of hipodensity of the encephalic parenquima on the right frontal region. Cerebrospinal fluid (CSF) puncture was executed for cytological, biochemistry and microbiologic analysis. A wide CSF viral screening panel was carried out by the Clinical Virology Laboratory. The assay was performed by real-time amplification reaction (PCR) using the ELITe InGeniusTM (combining extraction and an amplification thermocycler) and revealed the amplification of JC polyomavirus DNA. The remaining viruses were undetectable. Regarding the JC polyomavirus, JCV ELITe MGB® kit product, which is a qualitative nucleic acids amplification assay, was performed. In combination with MRI that revealed alterations on the white substance (periventricular and subcortical frontal region), with minor expression in the temporal and parietal regions, the diagnosis of PML was established. After diagnosis, the patient’s immunosuppressive regimen was reduced. MMF was stopped and prednisolone reduced to 10mg/day. The patient remained stable and was discharged after one week. He is currently having regular clinical evaluations as an outpatient.