TABLE 2.
Posttransplant lymphoproliferative disorder case characteristics
| Transplant diagnosis | Age (y) | UIP on explant | EBV Serostatus | Immunosuppression at diagnosis | Time to diagnosis (mo) | Location | Histology | Peak EBV VL (copies/mL) | Treatment | Survival after diagnosis (mo) |
|---|---|---|---|---|---|---|---|---|---|---|
| IPF | 52 | Yes | D+/R+ | Tacro, Pred | 39 | Allograft lung | Monomorphic B cell, EBER+ | 100 000 | None | 0.4 |
| IPF | 51 | Yes | D+/R+ | Tacro, Evero, Hydro | 64 | Allograft lung | Monomorphic B cell, EBER+ | 3 850 000 | Rituximab | 3.7 |
| Cystic fibrosis | 23 | No | D+/R− | Tacro, Myco, Pred | 5 | Small bowel | Monomorphic B cell, EBER+ | 30 000 | Rituximab | 16.4 |
| Cystic fibrosis | 24 | No | D+/R− | Tacro, Pred | 6 | Allograft lung | Polymorphic B cell, EBER+ | 23 000 | Rituximab | 8.4 |
| IPF | 69 | Yes | D+/R+ | Tacro, Aza, Pred | 35 | Allograft lung | Polymorphic B cell, EBER+ | 380 | Rituximab | 26.2 |
| IPF | 65 | Yes | D+/R+ | Tacro, Hydro | 57 | Allograft lung | Smooth muscle tumor, EBER+ | 16 000 | Lobectomy | 21.2 |
| COPD | 67 | No | D+/R+ | Tacro, Evero, Hydro | 9 | Allograft lung | Monomorphic B cell, EBER+ | 1000 | Rituximab | 7.5 |
| Scleroderma | 50 | Yes | D+/R− | Tacro, Myco, Pred | 9 | Cerebellum | Monomorphic B cell, EBER+ | 580 | Methotrexate/Rituximab | 20.4 |
| Cystic fibrosis | 51 | No | D+/R− | Tacro, Myco, Pred | 4 | Small bowel, nasopharynx, liver | Monomorphic B cell, EBER+; Liver sarcoma, EBER+ | 9410 | Rituximab | 35.6 |
| IPF | 65 | Yes | D+/R− | Tacro, Myco, Pred | 57 | Bone marrow, allograft lung | Monomorphic, peripheral T cell, EBER− | 1000 | None | 0.9 |
| Cystic fibrosis | 37 | No | D+/R− | Tacro, Myco, Pred | 4 | Tonsils | Polymorphic B cell, EBER+ | 8800 | R-CHOP | 14.8 |
| LAM | 40 | No | D+/R+ | Cyclo, Hydro, Aza | 6 | Allograft lung | Monomorphic B cell, EBER+ | Not tested | Rituximab | 56.9 |
| IPF | 73 | Yes | D+/R+ | Tacro, Hydro, Aza | 40 | Native lung, kidney, spleen, liver, bone marrow | Polymorphic B cell, EBER+ | 110 000 | None | 0 |
| IPF | 57 | Yes | D+/R+ | Tacro, Myco, Pred | 14 | Lung | Monomorphic B cell, EBER+ | 53 000 | R-CHOP | 6.6 |
| COPD | 67 | No | D+/R+ | Tacro, Myco, Pred, Evero | 24 | Brain, lungs, bone marrow, kidney, liver, spleen | Monomorphic B cell, EBER+ | 800 000 | None | 1.4 |
| COPD | 70 | No | D+/R+ | Tacro, Myco, Pred | 2 | Liver | Monomorphic B cell, EBER+ | 3300 | R-CHOP | 78.8 |
| COPD | 66 | No | D+/R+ | Belat, Myco, Pred | 15 | Liver | Monomorphic B cell, EBER+ | 12 000 | None | 9.6 |
| IPF | 62 | Yes | D+/R− | Tacro, Evero, Pred | 8 | Allograft lung | Monomorphic B cell, EBER+ | 100 000 | R-CHOP | 2 |
| IPF | 61 | Yes | D+/R− | Cyclo, Pred | 9 | Cerebrospinal fluid | Monomorphic B cell, EBER+ | 107 750 | Rituximab | 1.1 |
| Cystic fibrosis | 20 | No | D+/R− | Tacro, Myco, Pred | 3 | Small bowel | Mixed poly/monomorphic B cell, EBER+ | 96 000 | None | 0.1 |
| IPF | 65 | Yes | D+/R− | Tacro, Pred | 3 | Liver | Monomorphic DLBCL, EBER+ | 660 000 | Rituximab | 0.7 |
| IPF | 69 | Yes | D+/R− | Tacro, Pred | 4 | Allograft lung | Monomorphic B cell, EBER+ | 5 340 000 | Rituximab | 1.2 |
| COPD | 67 | No | D−/R+ | Cyclo, Pred | 7 | Allograft lung | Monomorphic B cell, EBER+ | 44000 | R-CHOP | 2.7 |
| Cystic fibrosis | 22 | No | D+/R− | Tacro, Myco, Pred | 2 | Allograft lung | Monomorphic B cell, EBER+ | 45000 | Rituximab | 10.2 |
| IPF | 51 | Yes | D+/R− | Tacro, Evero, Pred | 52 | Allograft lung | Monomorphic B cell, EBER+ | 190000 | Rituximab | 6.2 |
| Langerhans cell histiocytosis | 62 | No | D+/R− | Tacro, Evero, Pred | 23 | Tongue | Monomorphic B cell, EBER+ | 5200 | Rituximab | 6.3 |
| COPD | 51 | No | D−/R+ | Tacro, Aza, Pred | 41 | Colon | Monomorphic DLBCL, EBER+ | 13000 | Rituximab | 35.6 |
| COPD | 67 | No | D−/R+ | Tacro, Myco, Pred | 85 | Bone | Monomorphic DLBCL, EBER− | Not tested | R-CHOP | 7.6 |
All 28 cases of posttransplant lymphoproliferative disorder in the cohort are described.
Abbreviations: Aza, azathioprine; Belat, belatacept; COPD, chronic obstructive pulmonary disease; Cyclo, cyclosporine; DLBCL, diffuse large B cell lymphoma; D, donor; EBV, Epstein-Barr virus; EBER, EBV-encoded RNA; Evero, everolimus; Hydro, hydrocortisone; IPF, idiopathic pulmonary fibrosis; LAM, lymphangiomyomatosis; Myco, mycophenolic acid; Pred, prednisone; R, recipient; R-CHOP, rituximab plus cyclophosphamide, doxorubicin, vincristine, and prednisone; Tacro, tacrolimus; UIP, usual interstitial pneumonia.