Epstein–Barr virus‐positive monoclonal lymphoplasmacytic proliferation associated with neurosyphilis in an immunocompetent patient: A case report

Syphilis is an infectious disease caused by the spirochete bacterium Treponema pallidum. Neurosyphilis results from the infection of the nervous system with Treponema pallidum, which can occur at any stage of syphilis. Neurosyphilis is often overlooked because of its rarity. Early‐stage neurosyphili...

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Veröffentlicht in:Neuropathology 2024-04, Vol.44 (2), p.104-108
Hauptverfasser: Hibiya, Takashi, Nagahama, Kiyotaka, Matsumoto, Yoshie, Saito, Kuniaki, Sasaki, Nobuyoshi, Kobayashi, Keiichi, Otsu, Akiyasu, Shimasaki, Teppei, Takeuchi, Kengo, Shiokawa, Yoshiaki, Nagane, Motoo, Shibahara, Junji
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container_issue 2
container_start_page 104
container_title Neuropathology
container_volume 44
creator Hibiya, Takashi
Nagahama, Kiyotaka
Matsumoto, Yoshie
Saito, Kuniaki
Sasaki, Nobuyoshi
Kobayashi, Keiichi
Otsu, Akiyasu
Shimasaki, Teppei
Takeuchi, Kengo
Shiokawa, Yoshiaki
Nagane, Motoo
Shibahara, Junji
description Syphilis is an infectious disease caused by the spirochete bacterium Treponema pallidum. Neurosyphilis results from the infection of the nervous system with Treponema pallidum, which can occur at any stage of syphilis. Neurosyphilis is often overlooked because of its rarity. Early‐stage neurosyphilis with brain mass formation is rare. We present a case of early‐stage neurosyphilis with prominent Epstein–Barr virus (EBV)‐positive monoclonal lymphoplasmacytic proliferation in an immunocompetent patient. A 36‐year‐old man presented with a chief complaint of a progressively worsening headache, a newly developed skin rash, and a fever. Magnetic resonance imaging showed a mass lesion, which measured 18 mm in diameter, in the left frontal lobe of the cerebrum. The patient underwent an emergency operation to remove the abscess. A pathological investigation revealed complex findings. There was an abscess in the cerebrum. Lymphoplasmacytic meningitis was also noted. In addition, a vaguely nodular lesion, which was composed of plasmacytoid and lymphoid cells, was observed around the abscess. Immunohistochemically, an anti‐Treponema pallidum antibody revealed numerous Treponemas around the abscess. In situ hybridization revealed that the plasmacytoid and lymphoid cells were Epstein–Barr encoding region (EBER)‐positive; κ‐positive cells were significantly more prevalent than λ‐positive cells, suggesting light‐chain restriction. Postoperatively, parenteral antibiotics were administered for four weeks. The patient has been free of recurrence for two years since the surgery. No association between neurosyphilis and EBV‐positive lymphoplasmacytic proliferation has ever been reported. Mass formation in early‐stage neurosyphilis is an exceptionally rare event. The present case indicates that in syphilis patients, lymphoproliferative disorders that lead to mass formation may be caused by concomitant EBV reactivation. Furthermore, when treating patients with mass lesions of the central nervous system, it is important to check their medical history and perform laboratory screening for infectious diseases to avoid overlooking syphilis infections.
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Neurosyphilis results from the infection of the nervous system with Treponema pallidum, which can occur at any stage of syphilis. Neurosyphilis is often overlooked because of its rarity. Early‐stage neurosyphilis with brain mass formation is rare. We present a case of early‐stage neurosyphilis with prominent Epstein–Barr virus (EBV)‐positive monoclonal lymphoplasmacytic proliferation in an immunocompetent patient. A 36‐year‐old man presented with a chief complaint of a progressively worsening headache, a newly developed skin rash, and a fever. Magnetic resonance imaging showed a mass lesion, which measured 18 mm in diameter, in the left frontal lobe of the cerebrum. The patient underwent an emergency operation to remove the abscess. A pathological investigation revealed complex findings. There was an abscess in the cerebrum. Lymphoplasmacytic meningitis was also noted. In addition, a vaguely nodular lesion, which was composed of plasmacytoid and lymphoid cells, was observed around the abscess. Immunohistochemically, an anti‐Treponema pallidum antibody revealed numerous Treponemas around the abscess. In situ hybridization revealed that the plasmacytoid and lymphoid cells were Epstein–Barr encoding region (EBER)‐positive; κ‐positive cells were significantly more prevalent than λ‐positive cells, suggesting light‐chain restriction. Postoperatively, parenteral antibiotics were administered for four weeks. The patient has been free of recurrence for two years since the surgery. No association between neurosyphilis and EBV‐positive lymphoplasmacytic proliferation has ever been reported. Mass formation in early‐stage neurosyphilis is an exceptionally rare event. The present case indicates that in syphilis patients, lymphoproliferative disorders that lead to mass formation may be caused by concomitant EBV reactivation. 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In addition, a vaguely nodular lesion, which was composed of plasmacytoid and lymphoid cells, was observed around the abscess. Immunohistochemically, an anti‐Treponema pallidum antibody revealed numerous Treponemas around the abscess. In situ hybridization revealed that the plasmacytoid and lymphoid cells were Epstein–Barr encoding region (EBER)‐positive; κ‐positive cells were significantly more prevalent than λ‐positive cells, suggesting light‐chain restriction. Postoperatively, parenteral antibiotics were administered for four weeks. The patient has been free of recurrence for two years since the surgery. No association between neurosyphilis and EBV‐positive lymphoplasmacytic proliferation has ever been reported. Mass formation in early‐stage neurosyphilis is an exceptionally rare event. The present case indicates that in syphilis patients, lymphoproliferative disorders that lead to mass formation may be caused by concomitant EBV reactivation. 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Neurosyphilis results from the infection of the nervous system with Treponema pallidum, which can occur at any stage of syphilis. Neurosyphilis is often overlooked because of its rarity. Early‐stage neurosyphilis with brain mass formation is rare. We present a case of early‐stage neurosyphilis with prominent Epstein–Barr virus (EBV)‐positive monoclonal lymphoplasmacytic proliferation in an immunocompetent patient. A 36‐year‐old man presented with a chief complaint of a progressively worsening headache, a newly developed skin rash, and a fever. Magnetic resonance imaging showed a mass lesion, which measured 18 mm in diameter, in the left frontal lobe of the cerebrum. The patient underwent an emergency operation to remove the abscess. A pathological investigation revealed complex findings. There was an abscess in the cerebrum. Lymphoplasmacytic meningitis was also noted. In addition, a vaguely nodular lesion, which was composed of plasmacytoid and lymphoid cells, was observed around the abscess. Immunohistochemically, an anti‐Treponema pallidum antibody revealed numerous Treponemas around the abscess. In situ hybridization revealed that the plasmacytoid and lymphoid cells were Epstein–Barr encoding region (EBER)‐positive; κ‐positive cells were significantly more prevalent than λ‐positive cells, suggesting light‐chain restriction. Postoperatively, parenteral antibiotics were administered for four weeks. The patient has been free of recurrence for two years since the surgery. No association between neurosyphilis and EBV‐positive lymphoplasmacytic proliferation has ever been reported. Mass formation in early‐stage neurosyphilis is an exceptionally rare event. The present case indicates that in syphilis patients, lymphoproliferative disorders that lead to mass formation may be caused by concomitant EBV reactivation. Furthermore, when treating patients with mass lesions of the central nervous system, it is important to check their medical history and perform laboratory screening for infectious diseases to avoid overlooking syphilis infections.</abstract><cop>Melbourne</cop><pub>John Wiley &amp; Sons Australia, Ltd</pub><pmid>37424259</pmid><doi>10.1111/neup.12934</doi><tpages>5</tpages><orcidid>https://orcid.org/0000-0002-0018-1652</orcidid><orcidid>https://orcid.org/0000-0001-6243-139X</orcidid></addata></record>
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subjects abscess
Abscess - complications
Abscesses
Adult
Antibiotics
Cell Proliferation
Central nervous system
Cerebrum
Epstein-Barr virus
Epstein-Barr Virus Infections - complications
Epstein–Barr virus infections
Frontal lobe
Herpesvirus 4, Human
Humans
Hybridization
Immunocompetence
Immunoproliferative diseases
Infectious diseases
Lesions
Lymphocytes
Lymphoid cells
lymphoproliferative disorders
Magnetic resonance imaging
Male
Meningitis
Nervous system
Neuroimaging
Neurosyphilis
Neurosyphilis - complications
Neurosyphilis - diagnosis
Patients
Spirochetes
Syphilis
Syphilis - complications
Treponema pallidum
title Epstein–Barr virus‐positive monoclonal lymphoplasmacytic proliferation associated with neurosyphilis in an immunocompetent patient: A case report
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