diagnostic slide session case 2010-5
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Diagnostic Slide Session Case 2010-5. Sandra Camelo-Piragua 1 , Ronald A Goerss 2 and David N Louis 1 1 Pathology Department, Massachusetts General Hospital, Boston, MA 2 Pathology Department, South Miami Hospital, Miami , FL. DSS 2010-5. - PowerPoint PPT PresentationTRANSCRIPT
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Diagnostic Slide Session
Case 2010-5Sandra Camelo-Piragua1, Ronald A Goerss2 and David N Louis1
1 Pathology Department, Massachusetts General Hospital, Boston, MA2 Pathology Department, South Miami Hospital, Miami , FL
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DSS 2010-538-year-old man who presented with seizures in 2009, after a year of fluctuating neurological symptoms.
History of HIV, ongoing cocaine use and intermittent compliance with his anti-HIV regimen.
18 mo ago10 mo agoPresentationNormalLymphocytes (cells/ul)10128881890680-3280CD3+ (cells/ul)9217551474850-4950CD3+CD4+ (cells/ul)7136346350-1800CD3+CD8+ (cells/ul)8406841191250-2200Helper/suppressor ratio0.080.050.210.9-3.5HIV PCR-QN copies/ml1579102445803580
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Axial FLAIR (1-26-2009)
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H&E
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DIFFERENTIAL DIAGNOSIS
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Special stains:- AFB- GMS
Immunohistochemistry:- HSV1- HSV2- CMV- HHV8Ancillary StudiesNEGATIVE
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LHE
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Neurofilament
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SV-40
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CD3CD20
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CD3CD20
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CD10CD21BCL6BCL2
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KAPPA (ISH)LAMBDA (ISH)
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DiagnosisProgressive Multifocal Leukoencephalopathy (PML)Patient alive after one year of initial symptoms and radiologic abnormalityMarked and unusual inflammatory infiltrateImmune Reconstitution Inflammatory Syndrome (IRIS)-Marked inflammation (on/off anti-HIV therapy)-Differential diagnosis: lymphoma vs. reactive follicles
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ReferencesVendrely A, Bienvenu B, Gasnault J, Thiebault JB, Salmon D, Gray F. Fulminant inflammatory leukoencephalopathy associated with HAART-induced immune restoration in AIDS-related progressive multifocal leukoencephalopathy. Acta Neuropathol. 2005 Apr;109(4):449-55.
Cinque P, Bossolasco S, Brambilla AM et al. The effect of highly active antiretroviral therapy-induced immune reconstitution on development and outcome of progressive multifocal leukoencephalopathy: study of 43 cases with review of the literature. J Neurovirol. 2003; 9 Suppl 1:73-80
Hair LS, Nuovo G, Powers JM, Sisti MB, Britton CB, Miller JR. Progressive multifocal leukoencephalopathy in patients with human immunodeficiency virus. Hum Pathol. 1992 Jun;23(6):663-7
Miralles P, Berenguer J, Garcia de Viedma D, et al. Treatment of AIDS-associated progressive multifocal leukoencephalopathy with highly active antiretroviral therapy. AIDS. 1998; 12:2467-72
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DiagnosisProgressive Multifocal Leukoencephalopathy (PML)Unusual inflammatory infiltrate Hair LS et al. Hum Pathol, 1992Patient alive after one year of initial symptoms and radiologic abnormality.Immune Reconstitution Inflammatory Syndrome (IRIS)-Marked inflammation (On/Off HIV therapy)Diagnosis:Differential diagnosis:LymphomaReactive follicle formation
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Progressive Multifocal Leukoencephalopathy (PML)Demyelinating foci on the cortico-subcortical junction with microglial activation, astrocytosis, and lympho-plasmocytic infiltration
PML inclusions at the edge of the demyelinating lesion admixed with inflammation
Myelin destruction with focal necrosis and cavitation
- Unusual inflammatory infiltrate Hair LS et al. Hum Pathol, 1992
- Patient alive after one year of initial symptoms and radiologic abnormality
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Immune Reconstitution Inflammatory Syndrome (IRIS)Acute symptomatic or paradoxical deterioration of a presumed pre-existing infection that is temporarily related to recovery of the immune system
-Marked inflammation (On/Off HIV therapy)
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Immune Reconstitution Inflammatory Syndrome (IRIS)Diagnostic criteria:Patient with AIDS
HAART induced a decrease in HIV-1 VL and an increase in CD-4+ T lymphocytes
Symptoms consistent with an infection/inflammatory condition appeared while on retroviral therapy
- Symptoms could not be explained by a new acquired infection, the expected course of a previously recognized infection, or side effects of therapy
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PML + IRIS
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1-26-2009
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CortexWhite matter
*38 y.o*Axial FLAIR**T2 Coronal