Correspondence

Progressive multifocal leukoencephalopathy as the presenting feature of chronic lymphocytic leukaemia

A 70-year-old male retired hospital electrician, who had been previously well, presented with an 8-week progressive history of confusion, visual disturbance and fluctuating right upper limb weakness. A full blood count showed lymphocytosis with thrombocytopenia; haemoglobin 134 g/l, total white cell count 631 9 109/l, lymphocytes 612 9 109/l, neutrophils 13 9 109/l and platelet count 44 9 109/l with normal biochemistry. A human immunodeficiency virus (HIV) test was negative. Subsequent flow cytometry showed a clonal cell population positive for CD5, CD19, CD20, CD23, CD200 and surface IgM, negative for FMC7, CD79b, CD38 and CD10, consistent with a diagnosis of chronic lymphocytic leukaemia (CLL). T-cell subsets showed normal levels of CD4 (064 9 109/l) and CD8 (076 9 109/l) cells. Mild hypogammaglobulinemia was noted with IgG 539 g/l, IgA 042 g/l and IgM 01 g/l. The T2-weighted magnetic resonance imaging scan (Fig 1) showed symmetrical, confluent signal change within the supratentorial white matter, with little mass effect, involving subcortical U fibres (arrow) and splenium of corpus callosum (arrowhead). These findings were consistent with demyelination and, in an immunocompromised patient, suggestive of progressive multifocal leukoencephalopathy (PML). Cerebrospinal fluid (CSF) analysis showed normal protein and glucose levels with negative microscopy for organisms. Polymerase chain reaction (PCR) of the CSF was positive for John Cunningham (JC) virus, confirming the diagnosis of PML.

Progressive multifocal leukoencephalopathy is a rare demyelinating condition of the central nervous system that is frequently fatal, caused by reactivation of latent JC polyoma virus and associated with immune system suppression. It was first described in patients with CLL and Hodgkin lymphoma (Astrom et al, 1958). Lymphoproliferative disorders accounted for 60% of cases before the advent of acquired immunodeficiency syndrome (AIDS). The figure is now reported to be 13%, with AIDS accounting for 80% of cases (Koralnik et al, 2006). Progressive multifocal leukoencephalopathy may present with a variety of progressively deteriorating neurological symptoms, which include muscle weakness, cognitive, visual or gait abnormalities as well as sensory deficit or aphasia (Tan & Koralnik, 2010). In CLL it is more commonly associated with patients who have had treatment and there is also a suggestion of an association with purine analogues and haematopoietic stem cell transplants (Garcıa-Suarez et al, 2005). It is unclear if rituximab is definitely a risk factor for PML despite a comprehensive review of 57 cases (Carson et al, 2009). To establish a diagnosis of PML it is considered sufficient to detect JC virus DNA in the CSF together with typical neuroimaging findings. Ultrasensitive PCR techniques have a sensitivity of up to 95% (Berger et al, 2013). Occasionally it may be necessary to resort to a brain biopsy to establish a diagnosis. Currently there is no specific anti-retroviral drug used in PML and no satisfactory accepted treatment. Cytarabine, cidofovir and mirtazepine have been used empirically

Fig 1. T2-weighted magnetic resonance imaging scan of the brain. T2-weighted magnetic resonance imaging scan of the brain showing symmetrical, confluent signal change within the supratentorial white matter, involving subcortical U fibres (arrow) and splenium of corpus callosum (arrowhead).

570

ª 2014 John Wiley & Sons Ltd British Journal of Haematology, 2014, 167, 563–583

Correspondence although none has proved effective (Tan & Koralnik, 2010). The prognosis of PML in patients with a haematological malignancy is

Progressive multifocal leukoencephalopathy as the presenting feature of chronic lymphocytic leukaemia.

Progressive multifocal leukoencephalopathy as the presenting feature of chronic lymphocytic leukaemia. - PDF Download Free
109KB Sizes 0 Downloads 3 Views