1Department of Internal Medicine, Seoul National University Hospital, Seoul, Korea
2Department of Pathology, Samsung Medical Center, Seoul, Korea
3Department of Internal Medicine, Dong-A University College of Medicine, Busan, Korea
4Department of Oncology, Asan Medical Center, Seoul, Korea
5Department of Internal Medicine, Seoul National University Bundang Hospital, Seongnam, Korea
6Department of Internal Medicine, Yonsei University College of Medicine, Seoul, Korea
7Department of Internal Medicine, Korea University Anam Hospital, Seoul, Korea
8Department of Internal Medicine, Pusan National University Hospital, Busan, Korea
9Department of Medicine, Samsung Medical Center, Seoul, Korea
10Department of Internal Medicine, Soonchunhyang University Seoul Hospital, Seoul, Korea
Copyright © 2019 by the Korean Cancer Association
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Case No. | Sex | Age at diagnosis (yr) | Site of lymphoma involvement at diagnosis | HHV8 | ECOG PS | IPI score | Comorbidity | Treatmenta) | Response to chemotherapy | PFS (mo) | OS (mo) |
---|---|---|---|---|---|---|---|---|---|---|---|
1 | M | 70 | Pleural cavity | Positive | 1 | 1 | Lung cancer | 1st-line CHOP | PR | ≥ 6 | ≥ 11 |
2 | M | 67 | Pleural cavity, bone marrow, cervical lymph node | Positive | 1 | 4 | HBV | 1st-line CHOP | NA | ≥ 6 | ≥ 9 |
3 | M | 59 | Pleural cavity | Positive | 1 | 1 | HIV | No chemotherapy | NA | ≥ 4 | ≥ 14 |
4 | F | 78 | Pleural cavity | Positive | 2 | 4 | None | No chemotherapy | NA | 49 | 49 |
5 | M | 87 | Pleural cavity | Positive | 3 | NA | None | No chemotherapy | NA | ≥ 5 | ≥ 6 |
6 | M | 60 | Pleural cavity, pericardial cavity, peritoneal cavity | Positive | 2 | 3 | None | 1st-line CHOP | CR followed by PD | 28 | ≥ 173 |
2nd-line ICE → autoHSCT | PR | ||||||||||
7 | F | 59 | Vitreous body (eyeball) | Negative | 0 | 0 | HCV | 1st-line HD-MTX | CR | 66 | 66 |
8 | M | 80 | Pleural cavity, pericardial cavity | Negative | 3 | 5 | None | 1st-line R-CHOP | CR | ≥ 17 | ≥ 17 |
9 | M | 70 | Pericardial cavity | Negative | 1 | 3 | None | 1st-line R-CHOP | CR | ≥ 14 | ≥ 14 |
10 | M | 77 | Pleural cavity, peritoneal cavity | Negative | 2 | 5 | None | 1st-line R-CHOP | CR | ≥ 12 | ≥ 12 |
11 | M | 83 | Pleural cavity | Negative | 2 | 4 | None | 1st-line R-CVP | NA | ≥ 2 | ≥ 2 |
12 | M | 75 | Pleural cavity, pericardial cavity | Negative | 1 | 3 | None | 1st-line R-CHOP | CR | ≥ 46 | ≥ 49 |
13 | F | 76 | Pleural cavity | Unknown | 2 | 2 | None | 1st-line CHOP | CR | ≥ 5 | ≥ 5 |
14 | F | 73 | Pleural cavity, peritoneal cavity | Unknown | 1 | 4 | HBV | 1st-line CVP | PR | 10 | 10 |
15 | M | 86 | Pleural cavity | Unknown | 1 | 4 | None | 1st-line R-CVP | PR | ≥ 24 | ≥ 24 |
16 | F | 66 | Pleural cavity, peritoneal cavity | Unknown | 1 | 4 | None | 1st-line CHOP | CR | ≥ 99 | ≥ 99 |
17 | F | 39 | Pleural cavity, lung | Unknown | 1 | 3 | None | 1st-line R-CHOP | CR | ≥ 123 | ≥ 123 |
HHV8, human herpesvirus 8; ECOG PS, Eastern Cooperative Oncology Group performance status; IPI, international prognostic index; PFS, progression-free survival; OS, overall survival; CHOP, cyclophosphamide, doxorubicin, vincristine, and prednisolone; PR, partial remission; HBV, hepatitis B virus; NA, not assessed or not available; HIV, human immunodeficiency virus; ICE, ifosfamide, carboplatin, and etoposide; autoHSCT, autologous hematopoietic stem cell transplantation; CR, complete remission; PD, progressive disease; HCV, hepatitis C virus; HD-MTX, high-dose methotrexate; R-CHOP, rituximab plus CHOP; CVP, cyclophosphamide, vincristine, and prednisolone; R-CVP, rituximab plus CVP.
a) Drainage was performed if clinically indicated.
Characteristic | Entire cohort (n=17) | PEL (n=6) | HHV8-unrelated BCBL (n=6) | HHV8-unknown BCBL (n=5) |
---|---|---|---|---|
Age at diagnosis (yr) | 73 (39-87) | 68.5 (59-87) | 76 (59-83) | 73 (39-86) |
Sex | ||||
Male | 11/17 (64.7) | 5/6 (83.3) | 5/6 (83.3) | 1/5 (20.0) |
Female | 6/17 (35.3) | 1/6 (16.7) | 1/6 (16.7) | 4/5 (80.0) |
Site of lymphoma involvement | ||||
Pleural cavity | 15/17 (88.2) | 6/6 (100) | 4/6 (66.7) | 5/5 (100) |
Pericardial cavity | 4/17 (23.5) | 1/6 (16.7) | 3/6 (50.0) | 0/5 (0) |
Peritoneal cavity | 4/17 (23.5) | 1/6 (16.7) | 1/6 (16.7) | 2/5 (40.0) |
Extra-cavitary lesionb) | 2/17 (11.8) | 1/6 (16.7) | 0/6 (0) | 1/5 (20.0) |
ECOG PS at diagnosis | ||||
0-1 | 10/17 (58.8) | 3/6 (50.0) | 3/6 (50.0) | 4/5 (80.0) |
2-4 | 7/17 (41.2) | 3/6 (50.0) | 3/6 (50.0) | 1/5 (20.0) |
IPI risk group | ||||
Low (0-1) | 3/16 (18.8) | 2/5 (40.0) | 1/6 (16.7) | 0/5 (0) |
Low-intermediate (2) | 1/16 (6.2) | 0/5 (0) | 0/6 (0) | 1/5 (20.0) |
High-intermediate (3) | 4/16 (25.0) | 1/5 (20.0) | 2/6 (33.3) | 1/5 (20.0) |
High (4-5) | 8/16 (50.0) | 2/5 (40.0) | 3/6 (50.0) | 3/5 (60.0) |
Comorbidity | ||||
HIV | 1/17 (5.9) | 1/6 (16.7) | 0/6 (0) | 0/5 (0) |
HBV | 2/17 (11.8) | 1/6 (16.7) | 0/6 (0) | 1/5 (20.0) |
HCV | 1/17 (5.9) | 0/6 (0) | 1/6 (16.7) | 0/5 (0) |
Co-occurring malignancy | 1/17 (5.9)c) | 1/6 (16.7)c) | 0/6 (0) | 0/5 (0) |
Laboratory profile at diagnosis | ||||
WBC ≥ 10,000/μL | 3/17 (17.6) | 1/6 (16.7) | 0/6 (0) | 2/5 (40.0) |
Hemoglobin < 10 g/dL | 4/17 (23.5) | 1/6 (16.7) | 2/6 (33.3) | 1/5 (20.0) |
Platelet < (130×103)/μL | 1/17 (5.9) | 1/6 (16.7) | 0/6 (0) | 0/5 (0) |
Serum albumin < 3.5 g/dL | 12/17 (70.6) | 5/6 (83.3) | 5/6 (83.3) | 2/5 (40.0) |
Serum creatinine ≥ 1.5 mg/dL | 3/17 (17.6) | 1/6 (16.7) | 1/6 (16.7) | 1/5 (20.0) |
LDH above normal | 13/16 (81.2) | 4/6 (66.7) | 4/5 (80.0) | 5/5 (100) |
β2-microglobulin ≥ 3.5 mg/dL | 5/10 (50.0) | 2/4 (50.0) | 2/5 (40.0) | 1/1 (100) |
C-reactive protein ≥ 0.8 mg/dL | 17/17 (100) | 6/6 (100) | 6/6 (100) | 5/5 (100) |
Values are presented as median (range) or number (%). HHV8, human herpesvirus 8; PEL, primary effusion lymphoma; BCBL, body cavity-based lymphoma; ECOG PS, Eastern Cooperative Oncology Group performance status; IPI, international prognostic index; HIV, human immunodeficiency virus; HBV, hepatitis B virus; HCV, hepatitis C virus; WBC, white blood cell; LDH, lactate dehydrogenase.
a) The proportions are calculated as the frequency divided by the total number of evaluable patients for each characteristic,
b) Extra-cavitary lesions were present in one patient with PEL who had both bone-marrow and cervical lymph-node lesions and one patient with HHV8-unknown BCBL who had a lung lesion,
c) Lung cancer.
Values are presented as number (%). PEL, primary effusion lymphoma; HHV8, human herpesvirus 8; BCBL, body cavity-based lymphoma; CHOP, cyclophosphamide, doxorubicin, vincristine, and prednisolone; R-CHOP, rituximab plus CHOP; CVP, cyclophosphamide, vincristine, and prednisolone; R-CVP, rituximab plus CVP; HD-MTX, high-dose methotrexate.
Characteristic | Nador et al. [4] | Boulanger et al. [5] | Simonelli et al. [6] | Boulanger et al. [7] | Guillet et al. [8] | Present cohort |
---|---|---|---|---|---|---|
No. of cases | 15 | 12 | 11 | 28 | 34 | 6 |
Male-to-female ratio | 15:0 | 12:0 | 10:1 | 27:1 | 31:3 | 5:1 |
Age, median (range, yr) | 44 (31-85) | 43.5 (33-66) | 41 (26-58) | 44 (33-78) | 45 (40-54) | 68.5 (59-87) |
HIV positivity rate (%) | 87 | 100 | 100 | 100 | 100 | 17 |
Median survival | 5 mo | 5.6 mo | 6 mo | 6.2 mo | 10.2 mo | 4.1 yr |
Case No. | Sex | Age at diagnosis (yr) | Site of lymphoma involvement at diagnosis | HHV8 | ECOG PS | IPI score | Comorbidity | Treatment |
Response to chemotherapy | PFS (mo) | OS (mo) |
---|---|---|---|---|---|---|---|---|---|---|---|
1 | M | 70 | Pleural cavity | Positive | 1 | 1 | Lung cancer | 1st-line CHOP | PR | ≥ 6 | ≥ 11 |
2 | M | 67 | Pleural cavity, bone marrow, cervical lymph node | Positive | 1 | 4 | HBV | 1st-line CHOP | NA | ≥ 6 | ≥ 9 |
3 | M | 59 | Pleural cavity | Positive | 1 | 1 | HIV | No chemotherapy | NA | ≥ 4 | ≥ 14 |
4 | F | 78 | Pleural cavity | Positive | 2 | 4 | None | No chemotherapy | NA | 49 | 49 |
5 | M | 87 | Pleural cavity | Positive | 3 | NA | None | No chemotherapy | NA | ≥ 5 | ≥ 6 |
6 | M | 60 | Pleural cavity, pericardial cavity, peritoneal cavity | Positive | 2 | 3 | None | 1st-line CHOP | CR followed by PD | 28 | ≥ 173 |
2nd-line ICE → autoHSCT | PR | ||||||||||
7 | F | 59 | Vitreous body (eyeball) | Negative | 0 | 0 | HCV | 1st-line HD-MTX | CR | 66 | 66 |
8 | M | 80 | Pleural cavity, pericardial cavity | Negative | 3 | 5 | None | 1st-line R-CHOP | CR | ≥ 17 | ≥ 17 |
9 | M | 70 | Pericardial cavity | Negative | 1 | 3 | None | 1st-line R-CHOP | CR | ≥ 14 | ≥ 14 |
10 | M | 77 | Pleural cavity, peritoneal cavity | Negative | 2 | 5 | None | 1st-line R-CHOP | CR | ≥ 12 | ≥ 12 |
11 | M | 83 | Pleural cavity | Negative | 2 | 4 | None | 1st-line R-CVP | NA | ≥ 2 | ≥ 2 |
12 | M | 75 | Pleural cavity, pericardial cavity | Negative | 1 | 3 | None | 1st-line R-CHOP | CR | ≥ 46 | ≥ 49 |
13 | F | 76 | Pleural cavity | Unknown | 2 | 2 | None | 1st-line CHOP | CR | ≥ 5 | ≥ 5 |
14 | F | 73 | Pleural cavity, peritoneal cavity | Unknown | 1 | 4 | HBV | 1st-line CVP | PR | 10 | 10 |
15 | M | 86 | Pleural cavity | Unknown | 1 | 4 | None | 1st-line R-CVP | PR | ≥ 24 | ≥ 24 |
16 | F | 66 | Pleural cavity, peritoneal cavity | Unknown | 1 | 4 | None | 1st-line CHOP | CR | ≥ 99 | ≥ 99 |
17 | F | 39 | Pleural cavity, lung | Unknown | 1 | 3 | None | 1st-line R-CHOP | CR | ≥ 123 | ≥ 123 |
Characteristic | Entire cohort (n=17) | PEL (n=6) | HHV8-unrelated BCBL (n=6) | HHV8-unknown BCBL (n=5) |
---|---|---|---|---|
Age at diagnosis (yr) | 73 (39-87) | 68.5 (59-87) | 76 (59-83) | 73 (39-86) |
Sex | ||||
Male | 11/17 (64.7) | 5/6 (83.3) | 5/6 (83.3) | 1/5 (20.0) |
Female | 6/17 (35.3) | 1/6 (16.7) | 1/6 (16.7) | 4/5 (80.0) |
Site of lymphoma involvement | ||||
Pleural cavity | 15/17 (88.2) | 6/6 (100) | 4/6 (66.7) | 5/5 (100) |
Pericardial cavity | 4/17 (23.5) | 1/6 (16.7) | 3/6 (50.0) | 0/5 (0) |
Peritoneal cavity | 4/17 (23.5) | 1/6 (16.7) | 1/6 (16.7) | 2/5 (40.0) |
Extra-cavitary lesion |
2/17 (11.8) | 1/6 (16.7) | 0/6 (0) | 1/5 (20.0) |
ECOG PS at diagnosis | ||||
0-1 | 10/17 (58.8) | 3/6 (50.0) | 3/6 (50.0) | 4/5 (80.0) |
2-4 | 7/17 (41.2) | 3/6 (50.0) | 3/6 (50.0) | 1/5 (20.0) |
IPI risk group | ||||
Low (0-1) | 3/16 (18.8) | 2/5 (40.0) | 1/6 (16.7) | 0/5 (0) |
Low-intermediate (2) | 1/16 (6.2) | 0/5 (0) | 0/6 (0) | 1/5 (20.0) |
High-intermediate (3) | 4/16 (25.0) | 1/5 (20.0) | 2/6 (33.3) | 1/5 (20.0) |
High (4-5) | 8/16 (50.0) | 2/5 (40.0) | 3/6 (50.0) | 3/5 (60.0) |
Comorbidity | ||||
HIV | 1/17 (5.9) | 1/6 (16.7) | 0/6 (0) | 0/5 (0) |
HBV | 2/17 (11.8) | 1/6 (16.7) | 0/6 (0) | 1/5 (20.0) |
HCV | 1/17 (5.9) | 0/6 (0) | 1/6 (16.7) | 0/5 (0) |
Co-occurring malignancy | 1/17 (5.9) |
1/6 (16.7) |
0/6 (0) | 0/5 (0) |
Laboratory profile at diagnosis | ||||
WBC ≥ 10,000/μL | 3/17 (17.6) | 1/6 (16.7) | 0/6 (0) | 2/5 (40.0) |
Hemoglobin < 10 g/dL | 4/17 (23.5) | 1/6 (16.7) | 2/6 (33.3) | 1/5 (20.0) |
Platelet < (130×103)/μL | 1/17 (5.9) | 1/6 (16.7) | 0/6 (0) | 0/5 (0) |
Serum albumin < 3.5 g/dL | 12/17 (70.6) | 5/6 (83.3) | 5/6 (83.3) | 2/5 (40.0) |
Serum creatinine ≥ 1.5 mg/dL | 3/17 (17.6) | 1/6 (16.7) | 1/6 (16.7) | 1/5 (20.0) |
LDH above normal | 13/16 (81.2) | 4/6 (66.7) | 4/5 (80.0) | 5/5 (100) |
β2-microglobulin ≥ 3.5 mg/dL | 5/10 (50.0) | 2/4 (50.0) | 2/5 (40.0) | 1/1 (100) |
C-reactive protein ≥ 0.8 mg/dL | 17/17 (100) | 6/6 (100) | 6/6 (100) | 5/5 (100) |
Regimen | Entire cohort (n=17) | PEL (n=6) | HHV8-unrelated BCBL (n=6) | HHV8-unknown BCBL (n=5) |
---|---|---|---|---|
CHOP | 5 (29.4) | 3 (50.0) | 0 | 2 (40.0) |
R-CHOP | 5 (29.4) | 0 | 4 (66.7) | 1 (20.0) |
CVP | 1 (5.9) | 0 | 0 | 1 (20.0) |
R-CVP | 2 (11.8) | 0 | 1 (16.7) | 1 (20.0) |
HD-MTX | 1 (5.9) | 0 | 1 (16.7) | 0 |
No chemotherapy | 3 (17.6) | 3 (50.0) | 0 | 0 |
Characteristic | Nador et al. [4] | Boulanger et al. [5] | Simonelli et al. [6] | Boulanger et al. [7] | Guillet et al. [8] | Present cohort |
---|---|---|---|---|---|---|
No. of cases | 15 | 12 | 11 | 28 | 34 | 6 |
Male-to-female ratio | 15:0 | 12:0 | 10:1 | 27:1 | 31:3 | 5:1 |
Age, median (range, yr) | 44 (31-85) | 43.5 (33-66) | 41 (26-58) | 44 (33-78) | 45 (40-54) | 68.5 (59-87) |
HIV positivity rate (%) | 87 | 100 | 100 | 100 | 100 | 17 |
Median survival | 5 mo | 5.6 mo | 6 mo | 6.2 mo | 10.2 mo | 4.1 yr |
HHV8, human herpesvirus 8; ECOG PS, Eastern Cooperative Oncology Group performance status; IPI, international prognostic index; PFS, progression-free survival; OS, overall survival; CHOP, cyclophosphamide, doxorubicin, vincristine, and prednisolone; PR, partial remission; HBV, hepatitis B virus; NA, not assessed or not available; HIV, human immunodeficiency virus; ICE, ifosfamide, carboplatin, and etoposide; autoHSCT, autologous hematopoietic stem cell transplantation; CR, complete remission; PD, progressive disease; HCV, hepatitis C virus; HD-MTX, high-dose methotrexate; R-CHOP, rituximab plus CHOP; CVP, cyclophosphamide, vincristine, and prednisolone; R-CVP, rituximab plus CVP. Drainage was performed if clinically indicated.
Values are presented as median (range) or number (%). HHV8, human herpesvirus 8; PEL, primary effusion lymphoma; BCBL, body cavity-based lymphoma; ECOG PS, Eastern Cooperative Oncology Group performance status; IPI, international prognostic index; HIV, human immunodeficiency virus; HBV, hepatitis B virus; HCV, hepatitis C virus; WBC, white blood cell; LDH, lactate dehydrogenase. The proportions are calculated as the frequency divided by the total number of evaluable patients for each characteristic, Extra-cavitary lesions were present in one patient with PEL who had both bone-marrow and cervical lymph-node lesions and one patient with HHV8-unknown BCBL who had a lung lesion, Lung cancer.
Values are presented as number (%). PEL, primary effusion lymphoma; HHV8, human herpesvirus 8; BCBL, body cavity-based lymphoma; CHOP, cyclophosphamide, doxorubicin, vincristine, and prednisolone; R-CHOP, rituximab plus CHOP; CVP, cyclophosphamide, vincristine, and prednisolone; R-CVP, rituximab plus CVP; HD-MTX, high-dose methotrexate.
PEL, primary effusion lymphoma; HIV, human immunodeficiency virus. The five largest case series of PEL reported thus far (to our knowledge) were used for comparison.