Medicine2 - Myeloproliferative, Lymphoproliferative Workshop
PTLD. PTLD: Post-transplant Lymphoproliferative Disorders.
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Transcript of PTLD. PTLD: Post-transplant Lymphoproliferative Disorders.
PTLD
PTLD: Post-transplant Lymphoproliferative Disorders
PTLD: WHO (Jaffe, et al, 2001)
– Lymphoid proliferation or lymphoma that develops as a consequence of immunosuppression in a recipient of a solid organ or bone marrow allograft.
– PTLDs comprise a spectrum ranging from early EBV-driven polyclonal proliferations resembling infectious mononucleosis to EBV(+) or EBV(-) lymphomas of predominantly B-cell or less often T-cell type.
Post-transplant Lymphoproliferative Disorders- PTLD
• Early lesions
• Polymorphic PTLD
• Monomorphic PTLD (Classify according to lymphoma classification)
• Hodgkin lymphoma and Hodgkin-like PTLD
1. Early lesions
• Reactive plasmacytic hyperplasia
• Infectious Mononucleosis-like
• Possibly these are overlapping syndromes.
Infectious mononucleosis-like
Infectious mononucleosis-like, cont.
Infectious mononucleosis-like, cont.
Infectious mononucleosis-like, cont.
Infectious mononucleosis-like, cont.
1. Early lesions: Characteristics
• Lymphoid proliferation with preservation of architecture of the involved tissue.
• Younger age, solid organ transplants
• EBV + (If negative should not call PTLD)
• Polyclonal.
• Tend to regress with reduction in immunosuppression
• Can be fatal!
2. Polymorphic PTLD
15 Y/O F 125 days post unrelated donor cord transplant for MDS now with GI symptoms and Acute GVHD.
Jejunal Biopsy
2. Polymorphic PTLD, cont.
EBER
CD20
Polymorphic PTLD: Characteristics
• Destructive lesions composed of immunoblasts, plasma cells, and intermediate forms that efface the architecture.
• Full range of B-cell maturation.• Clonal proliferations.• EBV +• Some will regress with reduction in
immunosuppression.
3. Monomorphic PTLD
• B-cell neoplasms– Diffuse large B-cell lymphoma– Burkitt lymphoma– Myeloma– Plasmacytoma
• T-cell neoplasms– Peripheral T-cell lymphoma, NOS– Other types
3. Monomorphic PTLD: Case 1: DLBCL
Pt. E.K.: 72 Male 8 mos. post renal transplant now with inguinal lymphadenopathy.
3. Monomorphic PTLD:DLBCL
LMP1
CD20
Monomorphic PTLD: Case 2: DLBCL
Pt R.E.: 46 Y/O M 34 months post liver transplant now with lung and mediastinal masses.
Case 2: DLBCLCD 20
LMP1
Case 3: Myeloma
Pt. JS: 52 Y/O M 4 yrs post renal transplant now with pathologic fracture of left femur.
Lambda
LMP1
Monomorphic PTLD: Characteristics
• Can be diagnosed as lymphoma on morphologic grounds, and are classified accordingly (T- vs. B-cell).
• Most are EBV positive.
• Clonal proliferations.
• Tend to progress and require chemotherapy.
4. Hodgkin Lymphoma
• Mixed cellularity most common followed by
• Nodular sclerosis
• Lymphocyte depleted least common
Which PTLDs respond to reduction of immunosuppression?
• Morphology
• Immunophenotype
• EBV status
• Clonality
• Bcl-6 mutation: 3q27 t(3:22)(q27:q11) is associated with failure to regress in polymorphic and monomorphic PTLDs.