PMID- 37424276
OWN - NLM
STAT- MEDLINE
DCOM- 20240207
LR  - 20240207
IS  - 1440-1789 (Electronic)
IS  - 0919-6544 (Linking)
VI  - 44
IP  - 1
DP  - 2024 Feb
TI  - Pathology for severe inflammatory PML with PD1/PD-L1 expression of favorable 
      prognosis: What's a prognostic factor for PML-IRIS?
PG  - 47-58
LID - 10.1111/neup.12929 [doi]
AB  - A 72-year-old woman with dermatomyositis (DM) developed neurological 
      manifestation, and magnetic resonance imaging (MRI) revealed multiple 
      T2/fluid-attenuated inversion recovery (FLAIR)-hyperintense lesions predominantly 
      in the deep white matter of the cerebral hemisphere. Punctate or linear contrast 
      enhancement was observed surrounding the T1-hypointense area. Multiple 
      T2/FLAIR-hyperintense lesions were aligned along with the corona radiata. 
      Malignant lymphoma was first suspected, and a brain biopsy was performed. 
      Pathological investigation suggested the provisional diagnosis of "suspicious of 
      malignant lymphoma." Owing to emergent clinical conditions, high-dose 
      methotrexate (MTX) therapy was conducted, and then T2/FLAIR-hyperintense lesions 
      were dramatically reduced. However, the diagnosis of malignant lymphoma was 
      concerning since multiplex PCR demonstrated clonal restriction of the Ig H gene 
      for B cells and TCR beta genes for T cells. Histopathology revealed the 
      infiltration of both CD4(+) and CD8(+) T cells, and the CD4(+) /CD8(+) ratio was 
      4.0. Moreover, prominent plasma cells were observed, in addition to CD20(+) B 
      cells. Atypical cells with enlarged nuclei were present, and they were not 
      hematopoietic but found as glial cells. JC virus (JCV) infection was verified 
      with both immunohistochemistry and in situ hybridization; the final diagnosis was 
      progressive multifocal leukoencephalopathy (PML). The patient was treated with 
      mefloquine and discharged. This case is informative in understanding the host 
      anti-viral response. Variable inflammatory cells were observed, including CD4(+) 
      and CD8(+) T cells, plasma cells, and a small amount of perivascular CD20(+) B 
      cells. PD-1 and PD-L1 expression was observed in lymphoid cells and macrophages, 
      respectively. PML with inflammatory reactions was thought fatal, and autopsy 
      cases of PML with immune reconstitution inflammatory syndrome (IRIS) demonstrated 
      excessive infiltration of only CD8(+) T cells. However, this case revealed 
      infiltration of variable inflammatory cells, and a favorable prognosis would be 
      expected under PD-1/PD-L1 immune-checkpoint regulation.
CI  - (c) 2023 The Authors. Neuropathology published by John Wiley & Sons Australia, Ltd 
      on behalf of Japanese Society of Neuropathology.
FAU - Shishido-Hara, Yukiko
AU  - Shishido-Hara Y
AD  - Department of Anatomic Pathology, Tokyo Medical University, Tokyo, Japan.
AD  - Department of Pathology and Applied Neurobiology, Kyoto Prefectural University of 
      Medicine, Kyoto, Japan.
FAU - Akimoto, Jiro
AU  - Akimoto J
AD  - Department of Neurosurgery, Tokyo Medical University, Tokyo, Japan.
FAU - Fukami, Shinjiro
AU  - Fukami S
AD  - Department of Neurosurgery, Tokyo Medical University, Tokyo, Japan.
FAU - Kohno, Michihiro
AU  - Kohno M
AD  - Department of Neurosurgery, Tokyo Medical University, Tokyo, Japan.
FAU - Matsubayashi, Jun
AU  - Matsubayashi J
AD  - Department of Anatomic Pathology, Tokyo Medical University, Tokyo, Japan.
FAU - Nagao, Toshitaka
AU  - Nagao T
AD  - Department of Anatomic Pathology, Tokyo Medical University, Tokyo, Japan.
LA  - eng
GR  - Grant-in-Aid for Scientific Research C/
PT  - Case Reports
DEP - 20230709
PL  - Australia
TA  - Neuropathology
JT  - Neuropathology : official journal of the Japanese Society of Neuropathology
JID - 9606526
RN  - 0 (B7-H1 Antigen)
RN  - 0 (Programmed Cell Death 1 Receptor)
SB  - IM
MH  - Aged
MH  - Female
MH  - Humans
MH  - B7-H1 Antigen
MH  - CD8-Positive T-Lymphocytes/pathology
MH  - *Leukoencephalopathy, Progressive Multifocal/pathology
MH  - *Lymphoma
MH  - Prognosis
MH  - Programmed Cell Death 1 Receptor
OTO - NOTNLM
OT  - JC virus
OT  - PD-1/PD-L1
OT  - immune reconstitution inflammatory syndrome (IRIS)
OT  - methotrexate (MTX)
OT  - progressive multifocal leukoencephalopathy (PML)
EDAT- 2023/07/10 06:42
MHDA- 2024/02/05 06:43
CRDT- 2023/07/10 02:54
PHST- 2023/05/24 00:00 [revised]
PHST- 2023/04/17 00:00 [received]
PHST- 2023/06/05 00:00 [accepted]
PHST- 2024/02/05 06:43 [medline]
PHST- 2023/07/10 06:42 [pubmed]
PHST- 2023/07/10 02:54 [entrez]
AID - 10.1111/neup.12929 [doi]
PST - ppublish
SO  - Neuropathology. 2024 Feb;44(1):47-58. doi: 10.1111/neup.12929. Epub 2023 Jul 9.