Primary Effusion Lymphoma: A Clinicopathological Study of 70 Cases
Abstract
:Simple Summary
Abstract
1. Introduction
2. Materials and Methods
2.1. Patients
2.2. Flow Cytometric Immunophenotyping
2.3. Histological Examination and Immunohistochemistry Analysis
2.4. Fluorescence In Situ Hybridization (FISH) Studies
2.5. Statistical Analysis
3. Results
3.1. Clinical Features
3.2. Morphology, Immunophenotype, and Cytogenetic Findings
3.3. Staging, Treatment, and Outcome
3.4. Clinicopathologic and Prognostic Difference between Patients with Effusion-Only versus with Extracavitary-Only PEL
3.5. Clinicopathologic and Prognostic Differences between PEL Patients with HIV versus without HIV Infection
4. Discussion
5. Conclusions
Author Contributions
Funding
Institutional Review Board Statement
Informed Consent Statement
Data Availability Statement
Conflicts of Interest
References
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Parameters | Results |
---|---|
Age, median (range) | 46 (26–91) |
Gender, N (%) Male Female | 67 (95.7%) 3 (4.3%) |
Immune status, N (%) HIV-positive 1 HIV-negative 2 Unknown | 56 (80%) 8 (11.4%) 6 (8.6%) |
CBC, N (%) Anemia Leukocytosis Leukopenia Thrombocytopenia | 25/30 (83.3%) 4/30 (13.3%) 5/30 (16.7%) 14/29 (48.2%) |
Disease presentation | |
Effusion only, N (%) 3 Pleural Pericardial Peritoneal | 35/70 (50.0%) 24/35 (68.6%) 8/35 (22.9%) 7/35 (20.0%) |
Extracavitary only, N (%) 4 Lymph node Gastrointestinal tract Perirenal Skin/buccal Pelvic Central nerve system Atrium | 27/70 (38.6%) 16/27 (59.3%) 8/27 (25.9%) 2/27 (7.5%) 2/27 (7.5%) 1/27 (3.7%) 1/27 (3.7%) 1/27 (3.7%) |
Effusion and extracavitary, N (%) 5 | 8/70 (11.4%) |
Treatment, N (%) EPOCH CHOP Other regimens 6 | 13/26 (50.0%) 8/26 (30.8%) 5/26 (19.2%) |
Outcome Follow-up months, median (range) Medium OS (months) Deceased, N (%) | 40.0 (0–96) 42.5 26/52 (50%) |
Markers | Overall | Effusion Only (n = 35) | Extracavitary Only (n = 27) | Both Effusion and Extracavitary (n = 8) | p-Value * |
---|---|---|---|---|---|
CD3 | 14/65 (21.5%) | 4/30 (13.3%) | 9/27 (33.3%) | 1/8 (12.5%) | 0.07 |
CD20 | 5/66 (7.6%) | 1/32 (3.1%) | 1/27 (3.7%) | 3/7 (42.9%) | 0.90 |
CD30 | 44/60 (73.3%) | 22/27 (81.5%) | 15/25 (60.0%) | 7/8 (87.5%) | 0.09 |
CD38 | 32/34 (94.1%) | 19/20 (95.0%) | 10/11 (90.9%) | 3/3 (100%) | 0.89 |
CD43 | 8/14 (57.1%) | 4/7 (57.1%) | 2/5 (40.0%) | 2/2 (100%) | 0.56 |
CD45 | 50/59 (84.7%) | 25/26 (96.2%) | 18/26 (69.2%) | 7/7 (100%) | 0.01 |
CD79a | 5/37 (13.5%) | 1/8 (12.5%) | 4/25 (16.0%) | 0/4 (0%) | 0.81 |
CD138 | 30/58 (51.7%) | 12/26 (46.1%) | 15/25 (60.0%) | 3/7 (42.9%) | 0.32 |
ALK-1 | 0/34 (0%) | 0/8 (0%) | 0/23 (0%) | 0/3 (0%) | NA |
EMA | 16/28 (57.1%) | 10/10 (100%) | 4/15(26.7%) | 2/3 (66.7%) | 0.0005 *** |
HHV8 | 70/70 (100%) | 35/35 (100%) | 27/27 (100%) | 8/8 (100%) | NA |
PAX5 ** | 1/26 (3.8%) | 1/9 (11.1%) | 0/15 (0%) | 0/2 (0%) | 0.38 *** |
MUM1 | 31/36 (86.1%) | 14/14 (100.0%) | 15/20 (72.2%) | 2/2 (100.0%) | 0.06 *** |
Ki67 ≥ 70% | 25/26 (96.1%) | 6/6 (100%) | 15/15 (100%) | 4/5 (80.0%) | NA |
EBER | 39/60 (65.0%) | 14/27 (51.9%) | 20/26 (76.9%) | 5/7 (71.4%) | 0.06 |
K/L restriction | 15/37 (40.5%) | 6/20 (30.0%) | 6/11 (54.5%) | 3/6 (50%) | 0.18 |
Parameters | Effusion Only (n = 35) | Extracavitary Only (n = 27) | Both Effusion and Extracavitary (n = 8) | p-Value * |
---|---|---|---|---|
Age (years) | 52 (32–91) | 42 (26–77) | 43 (36–50) | 0.001 |
Gender | 33 M; 2 F | 26 M; 1 F | 8 M; 0 F | 0.71 |
HIV-positive, N (%) | 24/35 (68.6%) | 24/27 (88.9%) | 8/8 (100%) | 0.06 |
Kaposi sarcoma, N (%) | 9/19 (47.0%) | 7/10 (70%) | 3/5 (60%) | 0.24 |
Elevated LDH, N (%) | 9/14 (64.3%) | 4/6 (66.7%) | 3/5 (60%) | 0.92 |
CBC, N (%) Anemia Thrombocytopenia Leukopenia | 16/18 (88.9%) 7/17 (41.2%) 4/18 (22.2%) | 6/9 (66.7%) 4/9 (44.4%) 1/9 (11.1%) | 3/3 (100%) 3/3 (100%) 0/3 (0%) | 0.16 0.87 0.48 |
Bone marrow involvement, N (%) | 0/10 (0%) | 0/9 (0%) | 0/4 (0%) | NA |
Gain of MYC/8q by FISH, N (%) | 3/3 (100%) | 1/2 (50.0%) | NA | 0.40 ** |
Treatment, N (%) CHOP EPOCH | 4/12 (33.3%) 4/12 (33.3%) | 2/9 (22.2%) 6/9 (66.7%) | 2/5 (40.0%) 3/5 (60.0%) | 0.58 0.13 |
Outcome F/U time, median (range) (months) Mortality rate, N (%) Median OS (months) | 48.0 (0–96) 16/26 (61.5%) 30 | 26.9 (0–76) 7/20 (35%) 38.0 | 54.1 (0–88) 3/6 (50%) 48 | 0.21 0.07 0.34 |
Parameters | HIV-Positive (n = 56) | HIV-Negative (n = 8) | p-Value |
---|---|---|---|
Age (years) | 45 (26–77) | 75 (51–88) | 0.005 |
Gender | 55 M; 1 F | 8 M; 0 F | 1.00 |
Disease presentation, N (%) | |||
Effusion only | 24/56 (42.9%) | 7/8 (87.5%) | 0.02 * |
Extracavitary only | 24/56 (42.9%) | 1/8 (12.5%) | 0.10 |
Both effusion and extracavitary | 8/56 (14.3%) | 0/8 (0%) | 0.58 ** |
Kaposi sarcoma, N (%) | 17/56 (30.4%) | 0/8 (0%) | 0.10 ** |
EBER-positive, N (%) | 36/48 (75.0%) | 3/8 (37.5%) | 0.03 * |
CBC, N (%) Anemia Thrombocytopenia Leukopenia | 17/25 (68.0%) 13/24 (54.2%) 6/25 (24.0%) | 6/6 (100%) 0/6 (0%) 0/6 (0%) | 0.30 ** 0.02 ** 0.31 ** |
Bone marrow involvement, N (%) | 0/18 (0%) | 0/5 (0%) | NA |
Gain of MYC/8q by FISH, N (%) | 3/4 (75%) | 1/1 (100.0%) | 1.00 |
Treatment, N (%) CHOP EPOCH | 7/21 (33.3%) 11/21 (52.4%) | 1/5 (20.0%) 2/5 (40.0%) | 0.56 0.62 |
Outcome F/U time, median (range) (months) Mortality rate, N (%) Median OS (months) | 40.0 (0–70) 21/42 (50.0%) 42.5 | 26.9 (0–96) 4/8 (50.0%) 6.8 | 0.33 1.0 0.57 |
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Hu, Z.; Pan, Z.; Chen, W.; Shi, Y.; Wang, W.; Yuan, J.; Wang, E.; Zhang, S.; Kurt, H.; Mai, B.; et al. Primary Effusion Lymphoma: A Clinicopathological Study of 70 Cases. Cancers 2021, 13, 878. https://doi.org/10.3390/cancers13040878
Hu Z, Pan Z, Chen W, Shi Y, Wang W, Yuan J, Wang E, Zhang S, Kurt H, Mai B, et al. Primary Effusion Lymphoma: A Clinicopathological Study of 70 Cases. Cancers. 2021; 13(4):878. https://doi.org/10.3390/cancers13040878
Chicago/Turabian StyleHu, Zhihong, Zenggang Pan, Weina Chen, Yang Shi, Wei Wang, Ji Yuan, Endi Wang, Shanxiang Zhang, Habibe Kurt, Brenda Mai, and et al. 2021. "Primary Effusion Lymphoma: A Clinicopathological Study of 70 Cases" Cancers 13, no. 4: 878. https://doi.org/10.3390/cancers13040878