PTLD

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PTLD
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PTLD Post-transplant Lymphoproliferative
Disorders
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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.

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Post-transplant Lymphoproliferative Disorders-
PTLD

• Early lesions
• Polymorphic PTLD
• Monomorphic PTLD (Classify according to lymphoma
  classification)
• Hodgkin lymphoma and Hodgkin-like PTLD

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1. Early lesions

• Reactive plasmacytic hyperplasia
• Infectious Mononucleosis-like
• Possibly these are overlapping syndromes.

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Infectious mononucleosis-like
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Infectious mononucleosis-like, cont.
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Infectious mononucleosis-like, cont.
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Infectious mononucleosis-like, cont.
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Infectious mononucleosis-like, cont.
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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!

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2. Polymorphic PTLD
Jejunal Biopsy
15 Y/O F 125 days post unrelated donor cord
transplant for MDS now with GI symptoms and
Acute GVHD.
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2. Polymorphic PTLD, cont.
CD20
EBER
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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.

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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

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3. Monomorphic PTLD Case 1 DLBCL
Pt. E.K. 72 Male 8 mos. post renal transplant
now with inguinal lymphadenopathy.
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3. Monomorphic PTLDDLBCL
CD20
LMP1
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Monomorphic PTLD Case 2 DLBCL
Pt R.E. 46 Y/O M 34 months post liver transplant
now with lung and mediastinal masses.
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Case 2 DLBCL
CD 20
LMP1
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Case 3 Myeloma
LMP1
Lambda
Pt. JS 52 Y/O M 4 yrs post renal transplant now
with pathologic fracture of left femur.
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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.

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4. Hodgkin Lymphoma

• Mixed cellularity most common followed by
• Nodular sclerosis
• Lymphocyte depleted least common

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Which PTLDs respond to reduction of
immunosuppression?

• Morphology
• Immunophenotype
• EBV status
• Clonality
• Bcl-6 mutation 3q27 t(322)(q27q11) is
  associated with failure to regress in polymorphic
  and monomorphic PTLDs.