Table 3. Management of rare subtypes of... | American Society of Hematology

Table 3.

Management of rare subtypes of PTLD

PTLD subtype	Associated pathology	Treatment options	Additional considerations
Hodgkin PTLD	Most are EBV-related Large Reed Sternberg cells may be positive for CD20 and CD79a; however CD15 is frequently negative	Hodgkin-like treatments ABVD Brentuximab-AVD use with caution due to increased risk of infectious complications Rituximab may be considered for CD20⁺ disease	Worse prognosis than non-SOT–related cHL Use of checkpoint blockade associated with organ rejection and death (use with extreme caution)
Primary CNS lymphoma	May present as mPTLD or pPTLD, and these entities do not correlate with prognosis Three subtypes: Sporadic PCNS-LBCL–like: MYD88 and CD79B mutations are common Systemic RAS–driven type: extra-CNS involvement EBV-driven CNS-limited type: no oncogenic alterations	Rituximab plus high-dose methotrexate (>1.5 g/m²) Rituximab plus high-dose cytarabine (1 g/m²) Whole brain radiotherapy	Occurs most frequently after kidney SOT Kidney transplant is not an absolute contraindication for methotrexate
Plasmablastoid DLBCL	100% MUM1/IRF4⁺ 82% CD138⁺ 64% CD30⁺ 55% EBER⁺ Most have MYC and chromosome 17/TP53 derangements	In addition to treatment paradigm outlined in Figure 1, the addition of these may be considered: Brentuximab vedotin if CD30⁺ Daratumumab if CD138⁺ Bortezomib	Occasionally occurs after nonplasmablastoid PTLD
T-cell PTLD	Can present as any of the mature T-cell lymphoma subtypes Most common subtypes: Hepatosplenic T-cell lymphoma Primary cutaneous ALCL PTCL-NOS ALK- ALCL	Treat based on recommendations for each disease entity	Rare, ∼5% Often occurs as late event

PTLD subtype	Associated pathology	Treatment options	Additional considerations
Hodgkin PTLD	Most are EBV-related Large Reed Sternberg cells may be positive for CD20 and CD79a; however CD15 is frequently negative	Hodgkin-like treatments ABVD Brentuximab-AVD use with caution due to increased risk of infectious complications Rituximab may be considered for CD20⁺ disease	Worse prognosis than non-SOT–related cHL Use of checkpoint blockade associated with organ rejection and death (use with extreme caution)
Primary CNS lymphoma	May present as mPTLD or pPTLD, and these entities do not correlate with prognosis Three subtypes: Sporadic PCNS-LBCL–like: MYD88 and CD79B mutations are common Systemic RAS–driven type: extra-CNS involvement EBV-driven CNS-limited type: no oncogenic alterations	Rituximab plus high-dose methotrexate (>1.5 g/m²) Rituximab plus high-dose cytarabine (1 g/m²) Whole brain radiotherapy	Occurs most frequently after kidney SOT Kidney transplant is not an absolute contraindication for methotrexate
Plasmablastoid DLBCL	100% MUM1/IRF4⁺ 82% CD138⁺ 64% CD30⁺ 55% EBER⁺ Most have MYC and chromosome 17/TP53 derangements	In addition to treatment paradigm outlined in Figure 1, the addition of these may be considered: Brentuximab vedotin if CD30⁺ Daratumumab if CD138⁺ Bortezomib	Occasionally occurs after nonplasmablastoid PTLD
T-cell PTLD	Can present as any of the mature T-cell lymphoma subtypes Most common subtypes: Hepatosplenic T-cell lymphoma Primary cutaneous ALCL PTCL-NOS ALK- ALCL	Treat based on recommendations for each disease entity	Rare, ∼5% Often occurs as late event

ALCL, anaplastic large cell lymphoma; ALK-ALCL, ALK negative anaplastic large cell lymphoma; cHL, classical Hodgkin lymphoma; EBER, EBV-encoded RNA; PTCL-NOS, peripheral T-cell lymphoma-not otherwise specified; RAS, rat sarcoma.

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