Case Report

HHV8-Negative Primary Effusion Lymphoma of B-Cell Lineage: Two Cases and a Comprehensive Review of the Literature

Table 2

Detailed clinical characteristics of 48 cases of HHV8-negative effusion lymphomas of B-cell lineage.

Case Ref no.Age/sexOther diseaseHIVEBVHCVSites involvedMorphologyImmunophenotype#Molecular genetics/cytogeneticsTherapyOutcome

1Case 187/FCHF, AfibPleuraLargeCD19, CD45, CD20, CD79a * PleurodesisAlive 21 mo
2Case 282/FHTN, sick sinus syndrome, COPDPleuraLargeCD20, bcl-6, MUM1/IRF4, PAX5Clonal IGH@ Pleural effusion drainageDied 13 mo
3[5]99/F * Pleura, pericardiumMedium to large CD19, CD20, CD5, CD25, IgM, IgDMYC amplification but no rearrangement, Clonal IGH@ Pleural drainageAlive 16 mo
4[5]85/MHTN, AfibPleura, pericardiumMedium to large CD20Clonal IGH@, no MYC rearrangementNo treatmentAlive 11 mo
5[6]79/MHTN, CHFPleuraLarge pleomorphic CD45, CD20, CD79a, bcl-2, bcl-6, MUM1Clonal IGH@ Pleurodesis with doxycyclineAlive 55 mo
6[7]67/FRAPericardiumMedium to large CD20, CD79aClonal IGH@ CHOP and then followed by MEPP, DEVICDied 16 mo
7[8]74/M * PericardiumMedium to largeCD20 * Rituximab + CHOPDied 7 mo
8[9]63/MDM+Peritoneum, pleuraLarge pleomorphic CD19, CD20, CD22, CD45, HLA-DR, bcl-2, kappa Clonal IGH@ CHOPDied 5 mo
9[10]82/M * +Pleura.
pericardium
Medium to largeCD20, CD79a, Ig light chain restriction * CHOPAlive 18 mo
10[10]73/M * Pleura.
Pericardium, peritoneum
LargeCD20 * CHOPAlive 12 mo
11[11]77/MProstate ca, MI, idiopathic CD4+ T-cell lymphopenia+PleuraLargeCD45, CD19, CD20, CD79a, CD38, CD71, CD30, lambdaTrisomy 18. No rearrangements involving MYC, BCL2, BCL6, ALK and IGH CHOPlost to follow up
12[12]68/M *

PleuraLargeCD20, CD79aClonal IGH@. No MYC rearrangement R − CHOPAlive 22 mo
13[13]78/MIdiopathic CD4+ T-cell lymphopenia+
Pleura, pericardiumLargeCD19, CD20, CD22, HLA-DR, IgM, bcl-6Additional unknown material at 3q27 (BCL6). No MYC rearrangementR + THP-COPAlive 30 mo
14[4]88/MCAD

Pleura LargeCD20, CD30, CD79a, CD45 * R + CHOPAlive 11 mo
15[14]69/MNonePericardium, pleuraLarge pleomorphicCD19, CD20, CD5, kappa, bcl2, cyclin D1t(8; 14) (q24; q32); MYC-IGH rearrangement. Clonal IGH@ THP-COP Died 5 mo
16[15]52/F * * Pleura, pericardiumLarge pleomorphicCD19, CD20, CD22, CD45, HLA-DRClonal IGH@ * *
17[16]59/FHep C cirrhosis+PeritoneumSmall to medium-sizedCD20, CD10, IgG48,XX,t(8;22)(q24;q11), +16, +21; MYC-IGL rearrangement. Clonal IGH@ NoneDied 2 mo
18[17]57/M * + * PeritoneumMonomorphic, small to medium-sizedCD19, CD22, CD79a, CD10, CD23, CD38, IgM46,XY,t(8;22)(q24; q11); MYC-IGL rearrangement NoneDied 1 w
19[18]63/MHep C cirrhosis, HCC+PeritoneumMedium to large sizeCD19, CD20, CD22, IgG lambdaComplex karyotype with t(9;14). No MYC rearrangement. Clonal IGH@ NoneDied 22 mo
20[19]60/FCholesteatoma+PeritoneumLargeCD19, CD20, CD22, HLA-DRComplex karyotype including der(8) t(2;8) (q31;q24), but no MYC rearrangement identified by Southern blot NoneAlive 24 mo
21[20]65/MHep C cirrhosis+PeritoneumLargeCD19, CD20, CD22, IgH@Clonal IGH@. No MYC rearrangementPrednisolone, etoposideAlive 8 mo
22[21]65/MAlcoholic cirrhosis +PeritoneumLarge ImmunoblasticCD19, lambdaClonal IGH@ CHOPDied 12 mo
23[22]75/M * PleuraLargeCD19, CD20, HLA-DR, kappa Complex karyotype including MYC amplification. Clonal IGH@ CHOPDied 15 mo
24[22]76/M * * PleuraLarge CD19, CD20, CD10, HLA-DRComplex karyotype with t(8; 22)(q24,q11); Clonal IGH@ NoneAlive 6 mo
25[22]32/FCongenital protein-losing enteropathy * PeritoneumLargeCD19, CD20, CD10, HLA-DRComplex karyotype including MYC amplification. Clonal IGH@ CHOP, PBSCTAlive 13 mo
26[22]81/M * * PleuraLargeCD19, CD20, CD10, HLA-DR, CD5Complex karyotype including MYC amplification. Clonal IGH@ NoneAlive 2 mo
27[23]58/FDM, Hep C, hypothyroidism +PeritoneumLargeCD19, CD20, CD4, CD5Hyperdiploid karyotype including MYC rearrangement. Clonal IGH@ NoneDied 7 mo
28[24]58/FCVID+Pleura, pericardiumLargeCD19, CD20, CD22, HLA-DR, kappaNo MYC rearrangementPrednisoloneDied 18day
29[25]58/MHep C cirrhosis+PeritoneumLargeCD45, CD19, CD20, CD22, CD10, FMC7, HLA-DRClonal IGH@ CVPDied 5 mo
30[26]90/FAfibPleura peritoneum, pericardiumLargeCD20, CD79a, bcl-2MYC rearrangement. Clonal IGH@ NoneDied 5 mo
31[27]70/FNonePleura, pericardiumLargeCD19, CD20, CD22, CD24, CD8, CD10, HLA-DR, CD38Complex karyotype. No MYC rearrangement. Clonal IGH@ CHOP, SobuzoxaneAlive 30 mo
32[28]32/FLymphangioma, protein-losing enteropathy, chylothorax, Hep C+Pleura, peritoneumLargeCD19, CD20, CD10, HLA-DRComplex karyotype including MYC amplificationTHP-COP, PBSCTDied 18 mo
33[29]74/FHep C cirrhosis, allergic granulo-matous angiitis+Pleura, pericardium, peritoneumLargeCD45, CD19, CD20, CD25, HLA-DR, kappaNo MYC rearrangement. Clonal IGH@ Rituximab + THP-COPAlive 26 mo
34[30]75/FPericardiumLargeCD20, CD79at(1;22)(q21;q11), t(14;17)(q32;q23). No MYC rearrangement CHOPAlive 36 mo
35[31]90/MHistory of TBPleuraLargeCD19, CD20, CD30Complex karyotype including add(8)(q24). Clonal IGH@ Rituximab + THP-COPAlive 38 mo
36[31]87/F * PleuraLargeCD20, CD30, kappa * RituximabAlive 32 mo
37[32]74/MCKD, pulmonary infarction, DM * * * LargeCD19, CD20, MUM1, BLIMP1Clonal IGH@ Pleural effusion drainage Died 80 mo
38[32]87/MDM * * * Large immunoblasts typeCD19, CD20, MUM1Clonal IGH@ THP-CVP Died 16 mo
39[32]66/MDM, HTN, MI * * * Large CD19, CD20, MUM1Clonal IGH@ THP CVP + rituximabAlive 9 mo
40[32]94/FAfib * * * LargeCD20 * THP-CVPDied 1 mo
41[32]92/MCRF * * Medium to large sizedCD19, CD20, bcl-6, MUM1Clonal IGH@ No chemotherapy initially; THP-CVP 4 mo laterDied 9 mo
42[32]79/MDM, CRF * * * LargeCD19, CD20, MUM1Clonal IGH@ NoneAlive 7 mo
43[33]76/FHypothyroidism, pulmonary emphysema * * * Pleura, pericardiumMedium-sized monomorphic cellsCD19, CD20, CD21, surface Ig, HLA-DRX,Xq-,2q-,5q+,-6, +7p,+9p,+15,+r. Clonal IGH@ PrednisoloneDied 15 mo
44[34]55/MAutoimmune hemolytic anemia+PeritoneumLarge CD45, CD20, CD79a, CD38, IgM49,XY,add(3)(q11), der(8)t(1;8)(q12;p11),+r, +2mar. Clonal IGH@ CHOPDied 3 mo
45[20]65/FLiver cirrhosis, Hep C+PeritoneumLargeCD19, CD20, CD22No MYC rearrangementPrednisone, etoposideAlive 8 mo
46[35]92/FHTN, DM, ESRD * PleuraLargeCD20, CD45, bcl-2 * NoneDied 2 mo
47[36]70/MHep B, liver transplant+PleuraLargeCD19, CD20 * NoneAlive 8 mo
48[37]51/MNoneScrotumMedium to large sizeCD45, CD19, CD20, CD79aClonal IGH@ Carboplatin, etoposide, mitoxantrone, prednisone + radiotherapyAlive 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