Table 1 Clinicopathological features of primary effusion lymphoma in post-transplant patients
Ref/ age, sex, ethnicity/ | Serology/ possible source of HHV8 infection | Transplanted organ/time from TP to PEL diagnosis | IS therapy/HAART therapy | KS or other malignancies/pre-TP or post-TP | Site of PEL/BM involvement | Histology | IIC/EBER | Molecular data | Therapy survival from PEL diagnosis |
|---|---|---|---|---|---|---|---|---|---|
Jones 1998 59/M/Haitian | HIV-; no drug abuse; no sex with men | Heart/ 94 mos | AZA + CYA+ prednisone | KS (5 mos after TP) | Bilateral pleural effusions/BM NA | Large plasmacytoid cells, moderate basophilic cytoplasm, prominent multiple nucleoli | CD30+ EMA+ CD38+ CD10- CD19- CD20- CD79α- CD3- CD5- CD45RO- CD11b- CD13- CD14- CD33- CD45- CK- EBVLMP1- EBER- | IGH+,HHV8 DNA + EBVDNA- | CYA reduction Cycloph+ VCR+ prednisone (1cy). Bleo. CHOP (1cy). Ifo + eto (2 cy). Death 6 mos later. |
Dotti 1999 56/M/unknown | HCV+ HIV -HBV- | Heart/42 mos | AZA+ CYA + steroid | No | Peritoneal effusion BM positive at molecular analysis | Medium, large-sized cells, abundant basophilic cytoplasm, convoluted nuclei, large and multiple nucleoli | CD45+ CD38+ CD138+ HLA-DR+ CD30 + CD34- CD13-CD33- CD3-CD2- CD5-CD7- CD10-CD20- CD19-CD56- κ- λ- | IGH+ HHV8 DNA integration EBV genome integration 11q23 deletion No BCL6 c-MYC ALL-1 rearrangements. No Bcl2/IGH translocation. | CYA reduction, AZA stopping. No CT (for poor performance status). Death 1 mo later |
Boulanger 2008 57/M/African | HIV- HHV8+ (before TP) | Kidney/44 mos | TC+ MMF+ prednisone. 5 mos after TP: KS progression despite MMF stopping, TC and prednisone reduction+CT (dauno+ docetaxel+ bleo) 24mos after KS onset: TC stopping+ RAPA with partial KS remission. At PEL diagnosis RAPA blood level 12 ng/ml | Disseminated KS (5 mos after TP) | Right pleural effusion BM NA | PEL morphology | CD138+ CD38+ HHV8+ EBER- CD3- CD20- | IGH+ TCR-HHV8 DNA+ (PCR) Oligoclonal HHV8 episomes+ | CHOP + bleo (4 cys) Death 8 mos later |
Boulanger 2008 63/M/African | HIV- HHV8+ (before TP) | Kidney/54 mos | CYA+ MMF+ prednisone+ Anti CD25 moAb 3 mos after TP Meth (for rejection). 14 mos after TP CYA converted to RAPA (for renal graft impairment). At PEL diagnosis RAPA blood level 8.5 ng/ml | No | Cardiac tamponade BM NA | PEL morphology | CD30+ CD138+ HHV8+ EBER- CD20- CD3- | IGH- TCR-Oligoclonal HHV8 episomes+ | Cidofir Death 1 mo later |
Melo 2008 67/M/unknown | NA | Kidney/132 mos | AZA+ CYA+ prednisone | No | Left pleural effusion BM NA | Large hyperbasophilic cells with multiple nucleoli | CD45+ CD38+ CD138+ Negativity for B and T cells markers | HHV8+ EBV+ (PCR) | Cycloph+VCR + prednisone CY and AZA stopped RAPA (blood level 7 ng/ml) |
Testa 2010 55/M/unknown | HIV- HBV- HCV- HAV-CMV-HSV2-EBVDNA-HBVDNA- HCVRNA-CMVDNA-HSVDNA-HHV8- | Liver/12 mos | TC + MMF | No | Massive neoplastic infiltration of lungs, pleura, heart, IC muscles, around large vessels, Gerota fascia, omentum. Sclerosing peritonitis of stomach, intestine, liver BM NA | Large cells, prominent nucleoli, abundant basophilic cytoplasm | CD138+ EMA+ CD43+ vimentin+ HHV8+ kappa/lambda polyclonal ki67 > 90% CK- CD20- CD3- CD45- EBVLMP1- CD56- CD57- MPO- CD34- Melan-A- CD99- Desmin- CD68-TdT- CD30- calretinin- | HHV8DNA + EBVDNA- HSVDNA- CMVDNA- HHV6- | Diuretics Death 4 mos later Autopsy performed |
Rose 2012 42/M/Italian | HIV- HBV- HCV- | ASCT/276 mos | Myeloablative conditioning (Cicloph+TBI); chronic GVHD treated with CYA prednisolone, PUVA, THA. 24 mos after TP: pericarditis; THA stopped | No | Marked peritoneal effusion, small pleural effusion. Constrictive pericarditis BM NA | Atypical lymphoid cells | CD45+ CD138+ TdT- CD34- MPO- CD3- CD5- CD20- CD79α-CD30- ALK1-Ki67 100% | PCR: HHV8+ | PericardiectomyDeath 2 weeks later |
Shi 2012 60/M/Chinese | Serology pre-transplant: NA Serology at PEL diagnosis: HIV- HHV8+ | Kidney/120 mos | Long-term IS therapy (NS) | No | Bilateral pleural effusion, peritoneal effusion BM negative | Large cells with large nuclei, prominent nucleoli, scanty cytoplasm | HHV8+ CD30+ CD45+ vimentin+ EMA+ CD3- CD15- CD20- CD43- CD79α-CD45RO- CK-ALK1- EBVLMP1- | IGH+ IGL+ | CT (endoxan, farmorubicin, oncovin, prezolon). Death 4 mos later |
Christenson 2015 72/M/unknown | HIV- EBV DNA- CMV-No drug abuse; no sex with men; no link with HHV8 endemic areas | Liver/120 mos | TC (119 mos) changed to RAPA 1 mo prior PEL (due to altered renal function) | SCC of head and neck (surgery, CT, RT) | Pleural effusion BM NA | Large plasmacytoid cells, large convoluted nuclei, prominent nucleoli | CD45+ CD30+ CD38+ MUM1+ HHV8+ EBER - | NA | Rapamycin maintained+pleural drainage+intrapleural cidofovir. Due to cidofovir intolerance, bortezomib+Doxo. Death 7 mos later |
Kalogeraki 2015 49/M/unknown | HIV- | Kidney/340 mos | Prolonged IS therapy | No | Peritoneal effusion BM NA | Large cells with high N/C ratio, pleomorphic nuclei, prominent nucleoli, amphophilic cytoplasm | CD3+ CD138+ PAX5+ CD30+ CD45+/−HHV8+ (in situ hybridization) EBER-CK- EMA- CD2- CD5- CD19- CD20- CD43- CD79α- ALK1- TIA1-EBVLMP1- | IGH+ TCR+ | CHOP Alive at 10 mos |
Cain 2018 29/M/unknown | HIV + HBV- HCV – CMV- | Kidney/24 mos | TC + HAART | Nodal KS identified at autopsy | Pleural effusion lymph nodes spleen liver heart kidney lung BM NA | Intravascular large plasmacytoid and immunoblastic cells | CD45+ MUM1+ HHV8+ EBER+ CD20- CD79α- CD19 PAX5- CD2- CD3- CD5- CD7- CD4- CD8- ALK- MPO- CD138- kappa- lambda- IgM- IgD- CD34- CD117- CD30- EMA- CD10- Bcl6- TdT- | IGH and TCR NA for poor DNA quality | Death 2 weeks after admission Autopsy performed |
Kugasia 2018 63/M/Haitian | Remote history of polysubstance abuse Pre-transplant: HIV- EBV- HHV8- Post-transplant: HIV- EBV- HHV8+ | Heart/5 mos | TC+ prednisone | Cutaneous KS (pre-TP) treated with RT. KS recurrence at PEL diagnosis | Left pleural effusion BM positive | Large atypical cells with high N/C ratio, prominent nucleoli | CD45+ CD30+ HHV8+ CD138+ MUM1+ CD20- PAX5- CD4- CD8- CD56- ALK1- EBVLMP1- EMA- BCL2- BCL6- cyclin D1- CD10- calretinin -WT1- Ber-EP4- | NA | Cycloph, VCR, prednisone, brentuximab TC changed to sirolimus. (HHV8 undetectable) Death 14 mos later for acute graft rejection |
Zanelli 2019 42/M/Italian | HIV- HHV8- EBV- | Small bowel/7 mos | TC+ Anti-CD52 moAb | No | Multiple gastric and duodenal polyps BM negative | Large cells with eccentric nuclei and prominent nucleoli | CD138+ CD38+ CD30+ HHV8+ EBER+ Ki67 70% EMA+ lambda+ CD20- CD79α- PAX5- CD3- CD5- | NA | Death 1 mo later |