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Washington University Experience | INFECTION | Viruses | PML (JC Virus) | PML - Enigmatic Case | 1A0 Case 1 History
Case 1 History ---- The patient is a 62 year old male with history of recurrent Ehrlichia infections (6 years PTA and recently), anemia, positive ANA (1:320, for approximately 20 months) with butterfly facial rashes and recurrent mouth sores with negative lupus workup. He endorses intermittent fevers, chills and night sweats for the last few years in association with these symptoms. Rheumatologic workup (ESR>100) and treatment with Hydroxychloroquine failed to correct his symptoms and laboratory values. He developed progressive aphasia beginning 8 months prior to presentation which manifested as increasing difficulty with crossword puzzles and communicating and processing coding languages at work. He had not been previously tested for sexual transmitted infections and denied a history of sexually transmitted infection, penile discharge or genital ulcers. He endorsed a history of unprotected sex but not in the last few years. Notably, 9 months PTA he presented with hematuria and had a CT Urogram/bladder washing that was negative for high grade urothelial carcinoma. He had no history of organ transplant nor has he been an organ donor. He was treated with one immunomodulatory drug (hydroxychloroquine) in the last two years for possible seronegative RA but discontinued it after 1 year. He denied weight loss, headaches, changes in vision, hearing, ability to eat, focal weakness or numbness and lives and drives independently. The patient does not have recent travel history and does not have sick contacts. He denies tobacco use, significant alcohol use or recreational drug use. His family history is notable for cancer in his parents but his siblings are healthy. The patient presented 5 months ago with a transcortical motor aphasia affecting both spoken and written language with relative sparing of repetition. An MRI 4 months prior was concerning for low grade glioma in the setting of a left frontotemporal lesion. He had repeat imaging and subsequently had left temporal stereotactic brain biopsy that revealed (unexpected) progressive multifocal leukoencephalopathy. Following his PML diagnosis workup demonstrated active HIV with associated AIDS, and no other explanation for PML. His final diagnosis to date is transcortical aphasia secondary to HIV induced PML. The patient's CD4 count was 71. A CD4 count <200 & PML (an AIDS defining illness) is enough to diagnose him with AIDS. He was discharged on Biktarvy with careful followup with a concern for impending IRIS:
