Progressive Multifocal Leukoencephalopathy in Myelodysplastic Syndrome Involving Pure Red Cell Aplasia

Abstract

Progressive multifocal leukoencephalopathy (PML) is a rare and fatal demyelinating disease of the central nervous system caused by JC polyomavirus (JCV) reactivation in an immunocompromized host. We describe a case of PML in a 76-year-old woman with myelodysplastic syndrome, who had been treated with azathioprine for a pure red cell aplasia-like condition. PML was diagnosed based on the neurologic symptoms, the magnetic resonance imaging patterns and the detection of JCV DNA in the cerebrospinal fluid. She died ten months after the diagnosis. An autopsy confirmed the diagnosis, and JCV DNA was detected in the cerebrum. Azathioprine might have triggered PML.

Drug-associated progressive multifocal leukoencephalopathy: a clinical, radiological, and cerebrospinal fluid analysis of 326 cases

“…Apart from the intrinsically dismal prognosis of PML, this delay of several weeks to even months and additional assault on the immune system certainly may have contributed to the high mortality rate. Sometimes, acute symptoms and signs were attributed to cerebral infarctions [ 21 , 38 , 109 ]. Finally, ocular and vestibular symptoms have occasionally been misjudged as cataract and Meniere’s disease, respectively [ 45 , 120 , 155 ].…”

Signals of progressive multifocal leukoencephalopathy for immunosuppressants: a disproportionality analysis of spontaneous reports within the US Adverse Event Reporting System (AERS)

“…We also observed a signal for azathioprine and cyclosporine. For both drugs, there are case reports in which they were reported in the context of PML, although the USPIs of both drugs do not provide a warning with regard to PML. Recently, Mateen et al .…”

Isolated Isochromosome 17q in Myelodysplastic Syndromes with Pure Red Cell Aplasia and Basophilia

“…In contrast, MDS with PRCA and i(17q)/del(5q) may belong to the former type because these cases were correlated with high percentages of blasts (4,12,13). Treatment regimens for MDS with PRCA were varied: prednisolone, erythropoietin, cyclosporine, G-CSF, and azacitidine (9)(10)(11)(12)(13)(14)(15)(16)(17)(18)(19)(20)(21). Only one patient underwent allogeneic bone marrow transplantation after transformation to AML, and died at 57 months after the diagnosis (19).…”

“…Approximately 50 cases have been reported in the literature, although the definitions of erythroid hypoplasia were heterogeneous: erythroblasts were 1% or fewer, or less than 5% of bone marrow cells (9)(10)(11)(12)(13)(14)(15)(16)(17)(18)(19)(20)(21).…”