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8/22/2020 1 2020 Virtual Pathology Course Deniz Peker, MD Associate Professor Hematopathology 8/22/2020 “What makes it a lymphoma?” Deniz Peker, MD Disclosure No conflict of interest to disclose 1 2 3
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^t Z u l ] o Ç u Z } u M · 2020. 10. 5. · o ] l o o •/ v ( o u u } Ç l P } µ v •> P v ( } u ] u u µ v } o •, } P l ] v lZ r^ v P r o ] l o o •/ v ( o u u } Ç l P }

Oct 13, 2020

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Page 1: ^t Z u l ] o Ç u Z } u M · 2020. 10. 5. · o ] l o o •/ v ( o u u } Ç l P } µ v •> P v ( } u ] u u µ v } o •, } P l ] v lZ r^ v P r o ] l o o •/ v ( o u u } Ç l P }

8/22/2020

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2020 Virtual Pathology Course

Deniz Peker, MDAssociate Professor

Hematopathology

8/22/2020

“What makes it a lymphoma?”

Deniz Peker, MDDisclosure

• No conflict of interest to disclose

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8/22/2020

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Clinical History

89 year old male with history of Crohn’s disease was found to have multiple necrotic and ulcerating lesions in oral cavity

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• A lip biopsy was performed

Microscopy and Immunophenotype

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CD20

CD20

PAX5

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CD30

MUM1

EBER

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Diagnosis:EBV-associated lymphoproliferative disorder

What do you mean by $jfk#@?

Differential Diagnosis

1. EBV+ mucocutaneous ulcer

2. EBV+ diffuse large B-cell lymphoma, NOS

EBV+ Diffuse Large B-cell Lymphoma

EBV+ Mucocutaneous Ulcer

LocationSystemic nodal/extranodaldisease

Skin Mucosa (oral, GI)

Risk Factors Older age Immunosuppresion

Microscopy

• Large transformed immunoblasts

• Hodgkin/Reed-Sternberg-like cells

• Inflammatory background

• Large transformed immunoblasts• Hodgkin/Reed-Sternberg-like cells• Inflammatory background

Imuunophenotype

• CD19, CD20, PAX5, CD79a• MUM1• CD30• PDL1 and PDL2

• CD20, PAX5, OCT2• MUM1• CD30 and CD15*

Molecular Clonal Clonal or non-clonal

TreatmentChemo-immunotherapy Reduction of immunosuppression

RituximabLocal radiation

Prognosis Poor Good

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Clinical Information!

EBV+ MCU• No systemic symptoms

i.e. fever, night sweats, weight loss

• Localized lesion

EBV+ DLBCL• B-symptoms

• Systemic disease

EBV+ MUCOCUTANEOUS ULCER

• Age or immunosuppressant-related immunodeficiency

• Cutaneous and mucosal ulcers, no systemic disease

• Classical Hodgkin lymphoma, diffuse large B-cell lymphoma or monomorphic PTLD-like morphology and immunophenotype

• Angioinvasion and necrosis can be present

• EBER+; serum EBV titers not elevated (unlike PTLD)

• Good prognosis, often spontaneous regression with reduced immunosuppresants

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Take Home..

• Diagnostic line: “EBV+ lymphoproliferative disorder, see comment”

• Discuss likelihood of EBV+ MCU; BUT, include large B-cell lymphoma in the differential

• Patients need to be evaluated for systemic disease

THANK YOU

ISTANBUL

[email protected]

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