Progressive multifocal leukoencephalopathy
Progressive multifocal leukoencephalopathy, abbreviated PML, is a rare neuropathology caused by a polyomavirus.
General
- Caused by JC virus (a type of polyomavirus[1]) in the context of immunodeficiency; usu. in the setting of HIV infection.[2]
- Suspected cases are biopsied - unlike other demyelinating diseases.[4]
Gross
- Multifocal - as the name suggests.
DDx:
Microscopic
Features:[5]
- Perivascular inflammatory cells.
- Foamy histiocytes.
- Abnormal appearing glial cells:[6]
- Reactive astrocytes.
- Oligodendrocytes with nuclear enlargement and glassy magenta chromatin - key feature.
- Atypical mitoses - known as Creutzfeldt cell.
Note:
- The chromatin changes remind me of polyomavirus in urine cytology... perhaps not surprising as they are related.
Images
Oligodendrocytes with nuclear enlargement and glassy magenta chromatin (WC/jensflorian)
Same case with SV40-positive immunoreactivity (WC/jensflorian)
- PML - case 1 (upmc.edu).
- PML - case 1 (upmc.edu).
- Creutzfeldt cell - case 2 (upmc.edu).[7]
- PML oligodendrocyte (ucsf.edu).[6]
- PML oligodendrocyte (psychiatryonline.org).[8]
IHC
- SV40 +ve.[9]
See also
References
- ↑ Berger, JR. (Jun 2011). "The basis for modeling progressive multifocal leukoencephalopathy pathogenesis.". Curr Opin Neurol 24 (3): 262-7. doi:10.1097/WCO.0b013e328346d2a3. PMID 21499097.
- ↑ 2.0 2.1 Berger, JR. (2003). "Progressive multifocal leukoencephalopathy in acquired immunodeficiency syndrome: explaining the high incidence and disproportionate frequency of the illness relative to other immunosuppressive conditions.". J Neurovirol 9 Suppl 1: 38-41. doi:10.1080/13550280390195261. PMID 12709870.
- ↑ Mateen, FJ.; Muralidharan, R.; Carone, M.; van de Beek, D.; Harrison, DM.; Aksamit, AJ.; Gould, MS.; Clifford, DB. et al. (Aug 2011). "Progressive multifocal leukoencephalopathy in transplant recipients.". Ann Neurol 70 (2): 305-22. doi:10.1002/ana.22408. PMID 21823157.
- ↑ URL: http://path.upmc.edu/cases/case336/dx.html. Accessed on: 15 January 2012.
- ↑ URL: http://path.upmc.edu/cases/case120/dx.html. Accessed on: 3 January 2012.
- ↑ 6.0 6.1 URL: http://missinglink.ucsf.edu/lm/ids_104_Demyelination/Didactic/Pml.htm. Accessed on: 3 January 2012.
- ↑ URL: http://path.upmc.edu/cases/case336.html. Accessed on: 15 January 2012.
- ↑ Hurley, RA.; Ernst, T.; Khalili, K.; Del Valle, L.; Simone, IL.; Taber, KH. (2003). "Identification of HIV-associated progressive multifocal leukoencephalopathy: magnetic resonance imaging and spectroscopy.". J Neuropsychiatry Clin Neurosci 15 (1): 1-6. PMID 12556565.
- ↑ Muñoz-Mármol, AM.; Mola, G.; Fernández-Vasalo, A.; Vela, E.; Mate, JL.; Ariza, A. (Nov 2004). "JC virus early protein detection by immunohistochemistry in progressive multifocal leukoencephalopathy: a comparative study with in situ hybridization and polymerase chain reaction.". J Neuropathol Exp Neurol 63 (11): 1124-30. PMID 15581180.