Abstract:SUMMARY A case of cerebral mycosis fungoides co-existing with progressive multifocal leucoencephalopathy presented with dementia. Brain biopsy established the diagnosis of mycosis fungoides after cerebrospinal fluid examinations and computerised tomographic scanning of the brain produced non-specific abnormalities.Mycosis fungoides is a cutaneous lymphoma with characteristic histological and cytological features.' Visceral infiltration is a common necropsy finding and although the nervous system usually is spa… Show more
To determine some of the key clinical features that help prompt clinicians to pursue additional work-up for evaluation of CNS involvement of MF, we conducted a systematic review to better define characteristics, treatments, outcomes, and mortality in these patients. Our analyses indicated that neurologic surveillance after the diagnosis of MF is crucial. Review of systems should include change in mentation, vestibular, and ocular symptoms. Progression to CNS involvement does not always occur in tandem with cutaneous disease burden. Single-agent therapies can delay disease progression and improve prognosis. Multi-agent treatment does not improve survival.
To determine some of the key clinical features that help prompt clinicians to pursue additional work-up for evaluation of CNS involvement of MF, we conducted a systematic review to better define characteristics, treatments, outcomes, and mortality in these patients. Our analyses indicated that neurologic surveillance after the diagnosis of MF is crucial. Review of systems should include change in mentation, vestibular, and ocular symptoms. Progression to CNS involvement does not always occur in tandem with cutaneous disease burden. Single-agent therapies can delay disease progression and improve prognosis. Multi-agent treatment does not improve survival.
“…Moreover, the lack of effective JCV‐specific CD4 help may impair the functionality of anti‐JCV‐specific memory CD8 T cells 4 . Three cases of PML have previously been described with mycosis fungoides, a nonleukaemic CTCL; two of these patients received chemotherapy, the third had a cerebral metastatic infiltration 5–7 . PML cases in HIV seronegative patients have been reported in chronic lymphocytic leukaemia, and in patients receiving monoclonal antibodies such as natalizumab, rituximab or more recently efalizumab in psoriasis 8 .…”
Section: Proliferation Response Of the Patient’s Purified Cd4+ Peripmentioning
“…2 Symptoms arise as early as 4 months and as late as 30 years from the time of initial diagnosis, with mean time to onset described as 3.8 years to 7.5 years. 2,3 Pre-mortem diagnosis is usually made by cerebrospinal fluid (CSF) cytology, although cytology is often negative and the diagnosis is made at autopsy. 2,4 Brain biopsy is an alternative diagnostic method.…”
mentioning
confidence: 99%
“…2,4 Brain biopsy is an alternative diagnostic method. 3,5 We describe a patient who developed gait abnormalities, mental status changes, and brain magnetic resonance (MR) imaging abnormalities 6 years after the initial diagnosis of CTCL. Cytology was repeatedly negative, flow cytometry was inconclusive, and a brain biopsy was not performed because there was no discrete lesion for biopsy.…”
The authors describe a patient who was suspected of having cutaneous T cell lymphoma involvement of the brain despite repeatedly negative cerebrospinal fluid (CSF) cytology, inconclusive flow cytometry, and no discrete lesion for brain biopsy. The diagnosis was made by polymerase chain reaction (PCR) analysis that showed a monoclonal T cell receptor gamma-chain gene rearrangement in the CSF, identically sized to that present in a skin biopsy specimen. Thus, PCR could be used early and routinely to diagnose central nervous system spread of T cell lymphomas, because of its potentially superior sensitivity and specificity to CSF cytology.
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