Table 2.
Detailed clinical characteristics of 48 cases of HHV8-negative effusion lymphomas of B-cell lineage.
| Case | Ref no. | Age/sex | Other disease | HIV | EBV | HCV | Sites involved | Morphology | Immunophenotype# | Molecular genetics/cytogenetics | Therapy | Outcome |
|---|---|---|---|---|---|---|---|---|---|---|---|---|
| 1 | Case 1 | 87/F | CHF, Afib | — | — | — | Pleura | Large | CD19, CD45, CD20, CD79a | * | Pleurodesis | Alive 21 mo |
| 2 | Case 2 | 82/F | HTN, sick sinus syndrome, COPD | — | — | — | Pleura | Large | CD20, bcl-6, MUM1/IRF4, PAX5 | Clonal IGH@ | Pleural effusion drainage | Died 13 mo |
| 3 | [5] | 99/F | * | — | — | — | Pleura, pericardium | Medium to large | CD19, CD20, CD5, CD25, IgM, IgD | MYC amplification but no rearrangement, Clonal IGH@ | Pleural drainage | Alive 16 mo |
| 4 | [5] | 85/M | HTN, Afib | — | — | — | Pleura, pericardium | Medium to large | CD20 | Clonal IGH@, no MYC rearrangement | No treatment | Alive 11 mo |
| 5 | [6] | 79/M | HTN, CHF | — | — | — | Pleura | Large pleomorphic | CD45, CD20, CD79a, bcl-2, bcl-6, MUM1 | Clonal IGH@ | Pleurodesis with doxycycline | Alive 55 mo |
| 6 | [7] | 67/F | RA | — | — | — | Pericardium | Medium to large | CD20, CD79a | Clonal IGH@ | CHOP and then followed by MEPP, DEVIC | Died 16 mo |
| 7 | [8] | 74/M | * | — | — | — | Pericardium | Medium to large | CD20 | * | Rituximab + CHOP | Died 7 mo |
| 8 | [9] | 63/M | DM | — | + | — | Peritoneum, pleura | Large pleomorphic | CD19, CD20, CD22, CD45, HLA-DR, bcl-2, kappa | Clonal IGH@ | CHOP | Died 5 mo |
| 9 | [10] | 82/M | * | — | + | — | Pleura. pericardium |
Medium to large | CD20, CD79a, Ig light chain restriction | * | CHOP | Alive 18 mo |
| 10 | [10] | 73/M | * | — | — | — | Pleura. Pericardium, peritoneum |
Large | CD20 | * | CHOP | Alive 12 mo |
| 11 | [11] | 77/M | Prostate ca, MI, idiopathic CD4+ T-cell lymphopenia | — | + | — | Pleura | Large | CD45, CD19, CD20, CD79a, CD38, CD71, CD30, lambda | Trisomy 18. No rearrangements involving MYC, BCL2, BCL6, ALK and IGH | CHOP | lost to follow up |
| 12 | [12] | 68/M | * | — | — |
— |
Pleura | Large | CD20, CD79a | Clonal IGH@. No MYC rearrangement | R − CHOP | Alive 22 mo |
| 13 | [13] | 78/M | Idiopathic CD4+ T-cell lymphopenia | — | + | — |
Pleura, pericardium | Large | CD19, CD20, CD22, HLA-DR, IgM, bcl-6 | Additional unknown material at 3q27 (BCL6). No MYC rearrangement | R + THP-COP | Alive 30 mo |
| 14 | [4] | 88/M | CAD | — | — |
— |
Pleura | Large | CD20, CD30, CD79a, CD45 | * | R + CHOP | Alive 11 mo |
| 15 | [14] | 69/M | None | — | — | — | Pericardium, pleura | Large pleomorphic | CD19, CD20, CD5, kappa, bcl2, cyclin D1 | t(8; 14) (q24; q32); MYC-IGH rearrangement. Clonal IGH@ | THP-COP | Died 5 mo |
| 16 | [15] | 52/F | * | — | — | * | Pleura, pericardium | Large pleomorphic | CD19, CD20, CD22, CD45, HLA-DR | Clonal IGH@ | * | * |
| 17 | [16] | 59/F | Hep C cirrhosis | — | − | + | Peritoneum | Small to medium-sized | CD20, CD10, IgG | 48,XX,t(8;22)(q24;q11), +16, +21; MYC-IGL rearrangement. Clonal IGH@ | None | Died 2 mo |
| 18 | [17] | 57/M | * | — | + | * | Peritoneum | Monomorphic, small to medium-sized | CD19, CD22, CD79a, CD10, CD23, CD38, IgM | 46,XY,t(8;22)(q24; q11); MYC-IGL rearrangement | None | Died 1 w |
| 19 | [18] | 63/M | Hep C cirrhosis, HCC | — | − | + | Peritoneum | Medium to large size | CD19, CD20, CD22, IgG lambda | Complex karyotype with t(9;14). No MYC rearrangement. Clonal IGH@ | None | Died 22 mo |
| 20 | [19] | 60/F | Cholesteatoma | — | + | − | Peritoneum | Large | CD19, CD20, CD22, HLA-DR | Complex karyotype including der(8) t(2;8) (q31;q24), but no MYC rearrangement identified by Southern blot | None | Alive 24 mo |
| 21 | [20] | 65/M | Hep C cirrhosis | — | − | + | Peritoneum | Large | CD19, CD20, CD22, IgH@ | Clonal IGH@. No MYC rearrangement | Prednisolone, etoposide | Alive 8 mo |
| 22 | [21] | 65/M | Alcoholic cirrhosis | — | + | − | Peritoneum | Large Immunoblastic | CD19, lambda | Clonal IGH@ | CHOP | Died 12 mo |
| 23 | [22] | 75/M | * | — | − | − | Pleura | Large | CD19, CD20, HLA-DR, kappa | Complex karyotype including MYC amplification. Clonal IGH@ | CHOP | Died 15 mo |
| 24 | [22] | 76/M | * | — | − | * | Pleura | Large | CD19, CD20, CD10, HLA-DR | Complex karyotype with t(8; 22)(q24,q11); Clonal IGH@ | None | Alive 6 mo |
| 25 | [22] | 32/F | Congenital protein-losing enteropathy | — | − | * | Peritoneum | Large | CD19, CD20, CD10, HLA-DR | Complex karyotype including MYC amplification. Clonal IGH@ | CHOP, PBSCT | Alive 13 mo |
| 26 | [22] | 81/M | * | — | — | * | Pleura | Large | CD19, CD20, CD10, HLA-DR, CD5 | Complex karyotype including MYC amplification. Clonal IGH@ | None | Alive 2 mo |
| 27 | [23] | 58/F | DM, Hep C, hypothyroidism | — | − | + | Peritoneum | Large | CD19, CD20, CD4, CD5 | Hyperdiploid karyotype including MYC rearrangement. Clonal IGH@ | None | Died 7 mo |
| 28 | [24] | 58/F | CVID | — | + | − | Pleura, pericardium | Large | CD19, CD20, CD22, HLA-DR, kappa | No MYC rearrangement | Prednisolone | Died 18day |
| 29 | [25] | 58/M | Hep C cirrhosis | — | − | + | Peritoneum | Large | CD45, CD19, CD20, CD22, CD10, FMC7, HLA-DR | Clonal IGH@ | CVP | Died 5 mo |
| 30 | [26] | 90/F | Afib | — | − | − | Pleura peritoneum, pericardium | Large | CD20, CD79a, bcl-2 | MYC rearrangement. Clonal IGH@ | None | Died 5 mo |
| 31 | [27] | 70/F | None | — | − | − | Pleura, pericardium | Large | CD19, CD20, CD22, CD24, CD8, CD10, HLA-DR, CD38 | Complex karyotype. No MYC rearrangement. Clonal IGH@ | CHOP, Sobuzoxane | Alive 30 mo |
| 32 | [28] | 32/F | Lymphangioma, protein-losing enteropathy, chylothorax, Hep C | — | − | + | Pleura, peritoneum | Large | CD19, CD20, CD10, HLA-DR | Complex karyotype including MYC amplification | THP-COP, PBSCT | Died 18 mo |
| 33 | [29] | 74/F | Hep C cirrhosis, allergic granulo-matous angiitis | — | − | + | Pleura, pericardium, peritoneum | Large | CD45, CD19, CD20, CD25, HLA-DR, kappa | No MYC rearrangement. Clonal IGH@ | Rituximab + THP-COP | Alive 26 mo |
| 34 | [30] | 75/F | − | — | − | − | Pericardium | Large | CD20, CD79a | t(1;22)(q21;q11), t(14;17)(q32;q23). No MYC rearrangement | CHOP | Alive 36 mo |
| 35 | [31] | 90/M | History of TB | — | − | − | Pleura | Large | CD19, CD20, CD30 | Complex karyotype including add(8)(q24). Clonal IGH@ | Rituximab + THP-COP | Alive 38 mo |
| 36 | [31] | 87/F | * | — | − | − | Pleura | Large | CD20, CD30, kappa | * | Rituximab | Alive 32 mo |
| 37 | [32] | 74/M | CKD, pulmonary infarction, DM | * | — | * | * | Large | CD19, CD20, MUM1, BLIMP1 | Clonal IGH@ | Pleural effusion drainage | Died 80 mo |
| 38 | [32] | 87/M | DM | * | — | * | * | Large immunoblasts type | CD19, CD20, MUM1 | Clonal IGH@ | THP-CVP | Died 16 mo |
| 39 | [32] | 66/M | DM, HTN, MI | * | — | * | * | Large | CD19, CD20, MUM1 | Clonal IGH@ | THP CVP + rituximab | Alive 9 mo |
| 40 | [32] | 94/F | Afib | * | — | * | * | Large | CD20 | * | THP-CVP | Died 1 mo |
| 41 | [32] | 92/M | CRF | * | — | — | * | Medium to large sized | CD19, CD20, bcl-6, MUM1 | Clonal IGH@ | No chemotherapy initially; THP-CVP 4 mo later | Died 9 mo |
| 42 | [32] | 79/M | DM, CRF | * | — | * | * | Large | CD19, CD20, MUM1 | Clonal IGH@ | None | Alive 7 mo |
| 43 | [33] | 76/F | Hypothyroidism, pulmonary emphysema | * | * | * | Pleura, pericardium | Medium-sized monomorphic cells | CD19, CD20, CD21, surface Ig, HLA-DR | X,Xq-,2q-,5q+,-6, +7p,+9p,+15,+r. Clonal IGH@ | Prednisolone | Died 15 mo |
| 44 | [34] | 55/M | Autoimmune hemolytic anemia | — | + | — | Peritoneum | Large | CD45, CD20, CD79a, CD38, IgM | 49,XY,add(3)(q11), der(8)t(1;8)(q12;p11),+r, +2mar. Clonal IGH@ | CHOP | Died 3 mo |
| 45 | [20] | 65/F | Liver cirrhosis, Hep C | — | — | + | Peritoneum | Large | CD19, CD20, CD22 | No MYC rearrangement | Prednisone, etoposide | Alive 8 mo |
| 46 | [35] | 92/F | HTN, DM, ESRD | — | — | * | Pleura | Large | CD20, CD45, bcl-2 | * | None | Died 2 mo |
| 47 | [36] | 70/M | Hep B, liver transplant | — | + | — | Pleura | Large | CD19, CD20 | * | None | Alive 8 mo |
| 48 | [37] | 51/M | None | — | — | — | Scrotum | Medium to large size | CD45, CD19, CD20, CD79a | Clonal IGH@ | Carboplatin, etoposide, mitoxantrone, prednisone + radiotherapy | Alive 8 mo |
#Immunophenotype includes only positively expressed antigens. *Information not available or mentioned. Abbreviations: Afib: atrial fibrillation; ca: carcinoma; CAD: coronary artery disease; CHF: congestive heart failure; CHOP: cyclophosphamide, daunorubicin, oncovin, prednisolone; CKD: chronic kidney disease; COPD: chronic obstructive pulmonary disease; CRF: chronic renal failure; CVID: common variable immune deficiency; DEVIC: dexamethasone, etoposide, ifosfamide and carboplatin; DM: diabetes mellitus; ESRD: end-stage renal disease; HCC: hepatocellular carcinoma; Hep: hepatitis; HTN: hypertension; IGH@: immunoglobulin heavy chain gene rearrangement study; MEPP: mitoxantrone hydrochloride, etoposide, cisplatin and prednisolone; MI: myocardial infarction; mo: months; PBSCT: peripheral blood stem cell transplantation; R: rituximab; RA: rheumatoid arthritis; TB: tuberculosis; THP-COP: pirarubicin, cyclophosphamide, oncovin, prednisolone