Progressive multifocal leukoencephalopathy from JC virus in a patient with advanced mycosis fungoides

doi:10.1016/j.jaad.2007.06.025

Journal of the American Academy of Dermatology

Volume 57, Issue 5, November 2007, Pages 893-895

https://doi.org/10.1016/j.jaad.2007.06.025 Get rights and content

Progressive multifocal leukoencephalopathy is a central nervous system disease due to reactivation of the human polyoma JC virus in immunocompromised patients. Advanced mycosis fungoides patients are intrinsically immunosuppressed and susceptible to infections, but only rarely have been reported to develop progressive multifocal leukoencephalopathy. We report a case of progressive multifocal leukoencephalopathy developing in an advanced mycosis fungoides patient without prior history of immunosuppressive therapy.

Section snippets

Discussion

PML was first described in 1958 in immunosuppressed patients as a result of hematologic malignancies, antineoplastic therapies, organ transplantation, or certain inflammatory conditions.¹ PML occurs in up to 5% of patients with HIV and AIDS and, more recently, as a rare but serious adverse event during natalizumab treatment (a monoclonal antibody to alpha4-integrin that inhibits lymphocyte/monocyte adhesion to endothelium) for multiple sclerosis and Crohn's disease,¹ and during rituximab

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There are more references available in the full text version of this article.

Cited by (10)

The immunopathogenesis and immunotherapy of cutaneous T cell lymphoma: Pathways and targets for immune restoration and tumor eradication
2021, Journal of the American Academy of Dermatology
Citation Excerpt :
Oral vitamin D administration can upregulate cathelicidin production in atopic dermatitis, but this pathway is relatively unexplored in CTCL.10,11 Rarer infectious complications, such as progressive multifocal leukoencephalopathy, Pneumocystis jirovecii pneumonia, and toxoplasmosis, have also been observed in CTCL but do not dominate the picture to the same extent as bacterial and herpesvirus infections.7,12 One hypothesis is that microbial products may stimulate disease progression.
Cutaneous T cell lymphomas (CTCLs) are malignancies of skin-trafficking T cells. Patients with advanced CTCL manifest immune dysfunction that predisposes to infection and suppresses the antitumor immune response. Therapies that stimulate immunity have produced superior progression-free survival compared with conventional chemotherapy, reinforcing the importance of addressing the immune deficient state in the care of patients with CTCL. Recent research has better defined the pathogenesis of these immune deficits, explaining the mechanisms of disease progression and revealing potential therapeutic targets. The features of the malignant cell in mycosis fungoides and Sézary syndrome are now significantly better understood, including the T helper 2 cell phenotype, regulatory T cell cytokine production, immune checkpoint molecule expression, chemokine receptors, and interactions with the microenvironment. The updated model of CTCL immunopathogenesis provides understanding into clinical progression and therapeutic response.
Sézary syndrome: Immunopathogenesis, literature review of therapeutic options, and recommendations for therapy by the United States Cutaneous Lymphoma Consortium (USCLC)
2011, Journal of the American Academy of Dermatology
Citation Excerpt :
Another consequence of the decline in cytotoxic T-cell and NK cell functions is impaired activity against opportunistic infectious pathogens. A noticeable increase in severity of herpes viral infections in advanced SS and cases of progressive multifocal leukoencephalopathy as a result of polyomavirus26 have been reported among patients with SS who have never been treated with chemotherapeutics or other immune-suppressing agents. Defective neutrophil function as a result of the abnormal cytokine milieu may also account for enhanced severity of bacterial infections and, perhaps, for the increase in skin colonization with Staphylococcus aureus.
Sézary syndrome (SS) has a poor prognosis and few guidelines for optimizing therapy. The US Cutaneous Lymphoma Consortium, to improve clinical care of patients with SS and encourage controlled clinical trials of promising treatments, undertook a review of the published literature on therapeutic options for SS. An overview of the immunopathogenesis and standardized review of potential current treatment options for SS including metabolism, mechanism of action, overall efficacy in mycosis fungoides and SS, and common or concerning adverse effects is first discussed. The specific efficacy of each treatment for SS, both as monotherapy and combination therapy, is then reported using standardized criteria for both SS and response to therapy with the type of study defined by a modification of the US Preventive Services guidelines for evidence-based medicine. Finally, guidelines for the treatment of SS and suggestions for adjuvant treatment are noted.
Haemorrhagic cystitis and polyomavirus JC infection in ataxia telangiectasia
2009, Journal of Pediatric Urology
Citation Excerpt :
In this case, however, the second human polyomavirus, JCV, was detected by virus-specific PCR. JCV is mainly associated with central nervous system disease and only rarely found in kidney and bladder disease [8]. However, after primary infection viral genomic information persists lifelong in both the central nervous system and the urogenital tract.
We report the case of a young adolescent with ataxia telangiectasia (AT) and life-threatening haemorrhage from the bladder due to a combination of bladder wall telangiectasis, immunosuppressive therapy and an infection with polyomavirus JC. BK and JC are both members of the polyomavirus family. BK virus is a known cause of haemorrhagic cystitis in bone-marrow and nephropathy in kidney transplant patients, whereas JC virus is mainly associated with progressive multifocal leukoencephalopathy and only rarely found in haemorrhagic cystitis. Although opportunistic infections are uncommon in AT and virus replication was described as being down-regulated in ATM (AT mutated protein)-deficient cells, clinicians should be aware that severe haematuria in a patient with AT and undergoing immunosuppressive therapy is suggestive for polyomavirus JC-induced haemorrhagic cystitis.
Presence of Chlamydophila pneumoniae DNA in blood cells is a frequent event in patients with the late stage of primary cutaneous lymphomas and with atopic dermatitis
2018, Postepy Dermatologii i Alergologii
Cutaneous T-cell lymphoma
2017, Clinical and Basic Immunodermatology: Second Edition
Central nervous system infections in cancer patients and hematopoietic stem cell transplant recipients
2014, Cancer Treatment and Research

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: Dr Lee is currently affiliated with Boston University/Tufts University Dermatology Training Program.

: Funding sources: None.

: Conflicts of interest: None declared.

: Reprints not available from the authors.

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Case reportProgressive multifocal leukoencephalopathy from JC virus in a patient with advanced mycosis fungoides

Section snippets

Discussion

Blood

Progressive multifocal leukoencephalopathy revisited: has the disease outgrown its name?

Ann Neurol

Changes in the natural history of progressive multifocal leukoencephalopathy in HIV-negative lymphoproliferative disorders: impact of novel therapies

Am J Hematol

Progressive multiforce leukoencephalopathy in a lymphoma patient with complete remission after treatment with cytostatics and rituximab: case report and review of literature

Clin Neuropathol

Cellular and humoral immune response in progressive multifocal leukoencephalopathy

Ann Neurol

Case report
Progressive multifocal leukoencephalopathy from JC virus in a patient with advanced mycosis fungoides