Progressive multifocal leukoencephalopathy in a patient with silicosis

Bonnet, Alexandre; Kerbrat, Anne; Tattevin, Pierre; Salmon, Anne; Jouneau, S.; Edan, Gilles; Grill, Jacques

doi:10.1007/s00415-016-8216-y

Cited by 1 publication

(3 citation statements)

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“…While PML without overt immunosuppression has been described in the literature, it is considered an exceedingly rare entity. [3–44] However, our single-center retrospective review suggests that PML without overt immunosuppression may make up a substantial proportion of PML cases (11.8%). Of note, prior population-based studies also reported no obvious immunocompromising disease or treatment in 6.3% [38] and 6.5% [45] of PML patients.…”

Section: Discussionmentioning

confidence: 99%

“…A comprehensive literature review of PML in the absence of overt immunosuppression (Table 1) [3–44,46] showed that previously described cases (median age 61 [range 21–81] years; 26% female) were usually found to have underlying comorbidities that may be associated with some degree of immunosuppression, including idiopathic CD4 lymphocytopenia (17/79; 21.5%), chronic renal failure (7/79; 8.9%) [5,22,26] and liver disease (15/70; 19%) most commonly due to hepatitis (6/79; 7.6%) [5,10] or alcoholism (6/79; 7.6%). [5,7,38,43] While case 2 had significant alcohol consumption in the year prior to diagnosis, both laboratory (liver function tests, coagulation studies) and radiographic (computed tomography abdomen) testing did not yield evidence for liver dysfunction.…”

Section: Discussionmentioning

confidence: 99%

“…In fact, only 51/74 (68.9%) of reported cases had a fatal outcome, with the remaining patients surviving the disease with significant disability (Table 1). [3–44,46]…”

Section: Discussionmentioning

confidence: 99%

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Progressive multifocal leukoencephalopathy without overt immunosuppression

Jain,

Branstetter,

Savaram

et al. 2023

Medicine

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Progressive multifocal leukoencephalopathy (PML) is a central nervous system disease caused by the human polyomavirus 2 that usually occurs in a setting of immunodeficiency. PML without overt immunosuppression is considered a rare occurrence but has been described in multiple previous case reports and series. Its prevalence, overall frequency, and prognosis are largely unknown. This is a single-center retrospective review of all University of Florida cases with the ICD10 PML diagnosis code (A81.2). PML without overt immunosuppression was defined as absence of human immunodeficiency virus (HIV) infection, hematological malignancy, immunomodulatory/-suppressive medications, autoimmune conditions with a propensity for PML (sarcoidosis, systemic lupus erythematosus). Cases that did not fulfill criteria for clinically or histologically definite PML were excluded. Of 52 patients with the ICD10 code A 81.2, 17 fulfilled definite diagnostic criteria for PML. Overt immunosuppression was identified in 15/17 (88.2%) cases (10/17 (58.8%): human immunodeficiency virus; 5/17 (29.4%): immunomodulatory/-suppressive medication). Two/seventeen (11.8%) cases were consistent with PML without overt immunosuppression. Possible contributing factors were a preceding dog bite and mild hypogammaglobulinemia M (39 mg/dL) in case 1 and significant alcohol use without evidence for liver disease in case 2. Both cases were fatal within 6 (case 1) and 2 (case 2) months. The results suggest that PML without overt immunosuppression may be more common than previously described. Therefore, PML should be considered even in the absence of overt immunosuppression if clinical and radiographic findings are suggestive of the diagnosis.

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Section: Discussionmentioning

confidence: 99%