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Lymphoma - Non B cell neoplasms

Post-transplantation lymphoproliferative disorders

Monomorphic PTLD


Reviewer: Dragos Luca, M.D. (see Reviewers page)
Revised: 28 October 2011, last major update August 2011
Copyright: (c) 2001-2011, PathologyOutlines.com, Inc.

Definition
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● Fulfill the criteria for one of the B cell or NK/T cell neoplasms recognized in the immunocompetent host (WHO Classification of Tumours of Haematopoietic and Lymphoid Tissues, 4th Edition, Lyon 2008)
● Exception: small B cell lymphoid neoplasms (follicular lymphoma, MALT) not designated as a post-transplantation lymphoproliferative disorder (PTLD)
● Monomorphic-PTLD (M-PTLD) should be diagnosed as a PTLD and further subclassified based on the WHO lymphoma classification (e.g., post-transplant lymphoproliferative disorder, diffuse large B cell lymphoma type)
● Monoclonal transformed B lymphocytic or plasmacytic proliferations that fulfil the criteria for a diffuse large B cell lymphoma, less often a Burkitt lymphoma or a plasma cell neoplasm (Haematologica 2011;96:1067)

Clinical features
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● Similar to B cell lymphomas and plasma cell neoplasms in immunocompetent individuals

Case reports
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● 11 year old boy and 44 year old man with Burkitt-like PTLD (Pathol Oncol Res 2002;8:105)
● 38 year old woman with M-PTLD of ovary (J Med Case Reports 2010;4:184)
● 47 year old man with M-PTLD of tongue (Diagn Pathol 2007;2:49)

Micro description
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● Fulfill criteria for conventional diffuse large B cell lymphoma, Burkitt lymphoma, myeloma or plasmacytoma
● Lack the full range of maturation seen in polymorphic-PTLD
● Significant pleomorphism may be present; monomorphic does not mean complete cellular monotony

Micro images
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EBV+ tumor cells in transplant patient


EBV+ diffuse large B cell lymphoma and Burkitt lymphoma


EBER using chromogenic in situ hybridization

Immunophenotype
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● Non-plasmacytic lesions: CD19+, CD20+, CD79a+, PAX-5+, monotypic Ig (often γ or α heavy chain), CD30+ (many)
● Most M-PTLD are of non-germinal center type, based on immunohistochemistry
● EBV+ cases: CD10-, BCL6+/-, IRF4/MUM1+, CD138-/+ (late/post-GC phenotype)
● EBV- cases: CD10+/-, BCL6+, IRF4/MUM1-, CD138- (GC phenotype)

Genetics and molecular
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● Clonal Ig gene rearrangements in virtually all cases
● EBV genomes in clonal episomal form
● Somatic mutations of IGV
RAS, TP53, MYC, BCL6 anomalies
● Cytogenetic abnormalities: 1q11-q21, 8q24.1, 3q27, 16p13, 14q32, 11q23-24, +9, +11, +7, +X, +2, +12
● EBV- PTLD: often lack expression of CDKN2A


Monomorphic T/NK-cell PTLD
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● Fulfill the criteria for any of the T/NK-cell lymphomas, and includes almost the entire spectrum of these neoplasms with the largest group being peripheral T cell lymphoma-NOS, followed by hepatosplenic T cell lymphoma, but also including T-large granular lymphocytic leukemia, adult T cell leukemia/lymphoma, extranodal NK/T cell lymphoma-nasal type, mycosis fungoides/Sezary syndrome, primary cutaneous anaplastic large cell lymphoma

Clinical features
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● Up to 15% of PTLD in North America & Western Europe
● Presentation dependent on the particular type of T/NK-cell neoplasm
● Mostly extranodal presentation: perpiheral blood, bone marrow, spleen, skin, liver, GI tract, lung

Case reports
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● 20 year old man with primary cardiac T cell PTLD (J Clin Pathol 2007;60:447)
● 45 year old man with EBV+ T cell PTLD presenting as acute appendicitis (J Med Case Reports 2011;5:5)
● 68 year old man, post-heart transplant, with primary cutaneous anaplastic large cell lymphoma (Acta Derm Venereol 2009;89:74)

Micro description
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● Similar to T/NK-cell lymphomas in immunocompetent individuals
● Frequency: PTCL-NOS 35%, HSTL 13%, other types of TCL <10% each

Immunophenotype
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● Pan-T cell antigens, NK-antigens, CD4 or CD8, may have CD30 or ALK, αβ or γδ
● EBV+ in about 1/3 of cases

Genetics and molecular
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● Clonal T cell receptor gene rearrangements (cases of T cell origin)
● Chromosomal anomalies similar to their counterparts in immunocompetent hosts: i(7)(q10) & +8 (HSTL); also TP53 mutations

End of Lymphoma - Non B cell neoplasms > Post-transplantation lymphoproliferative disorders > Monomorphic PTLD


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