Comparison of the clinical, pathologic, and genetic features of PMBCL and related entities
| Clinical, pathologic, and genetic features . | PMBCL . | Nonmediastinal PMBCL signature-positive DLBCL . | MGZL . | NScHL . |
|---|---|---|---|---|
| Clinical and pathological features | ||||
| Female:Male | 2:1 | 1:1 | 1:3 | 1:1 |
| Median age (y) | ∼35 | ∼66 | ∼34-40 | ∼28 |
| Stages I and II, % | 70-80 | 56 | 65 | 55 |
| Mediastinal presentation, % | All | None | 100 | 80 |
| Bone marrow, % | Rare | 19 | Rare | Rare |
| Bulky disease (≥10 cm), % | 70-80 | 6 | 30 | 54 |
| Morphology | Sheets of large cells; clear cells; no inflammatory polymorphous infiltrate | Similar to DLBCL | Broad cytologic appearance; may resemble PMBCL (25%); cHL (25%); intermediate (45%); composite (5%) | Lacunar Hodgkin Reed-Sternberg cells; inflammatory polymorphous infiltrate |
| Sclerosis, % | 70-100 (alveolar, fine bands) | Negative | Variable | 100 (large bands, annular) |
| 5-year OS, % | ∼90-97 | 72 (2-year DSS)* | ∼75 | ∼85 |
| Immunohistochemistry, % | ||||
| CD45 | 100 | Not reported | Positive | Negative |
| CD30 | 70-85 (weak) | 31 (weak) | ∼85-100 (strong) | 100 |
| CD15 | Negative | Negative | 58-80 | 75-85 |
| CD20 CD79a PAX-5 MUM1 | 100 100 100 75 | 100 Not reported* Not reported 19 | ∼72-98 (weak) 67-75 98 Positive | Very rare (minority) Very rare 95 (weak) 100 |
| MAL CD23 | 60-70 85 | 57 71 | 30-49* 30-49 | 19 Negative |
| TRAF1 expression Nuclear cRel | 60-70 60-70 | Not reported Not reported | Usually positive Not reported | 84 92 |
| PDL1 PDL2 | 71 72 | 19 14 | ∼80 ∼30 | 87 |
| Genetic aberrations, % | ||||
| CIITA rearrangements 9p.24.1 aberrations (JAK2, PDL) 2p16.1 (Rel, BCL11A) SOCS1 mutation/deletion B2M mutations | 38 70 25-50 35-45 30-64 | Negative 33 Frequent 100 ∼15 | 30-37 60 33 40 32 | 15 25-30 25 42 40 |
| Clinical, pathologic, and genetic features . | PMBCL . | Nonmediastinal PMBCL signature-positive DLBCL . | MGZL . | NScHL . |
|---|---|---|---|---|
| Clinical and pathological features | ||||
| Female:Male | 2:1 | 1:1 | 1:3 | 1:1 |
| Median age (y) | ∼35 | ∼66 | ∼34-40 | ∼28 |
| Stages I and II, % | 70-80 | 56 | 65 | 55 |
| Mediastinal presentation, % | All | None | 100 | 80 |
| Bone marrow, % | Rare | 19 | Rare | Rare |
| Bulky disease (≥10 cm), % | 70-80 | 6 | 30 | 54 |
| Morphology | Sheets of large cells; clear cells; no inflammatory polymorphous infiltrate | Similar to DLBCL | Broad cytologic appearance; may resemble PMBCL (25%); cHL (25%); intermediate (45%); composite (5%) | Lacunar Hodgkin Reed-Sternberg cells; inflammatory polymorphous infiltrate |
| Sclerosis, % | 70-100 (alveolar, fine bands) | Negative | Variable | 100 (large bands, annular) |
| 5-year OS, % | ∼90-97 | 72 (2-year DSS)* | ∼75 | ∼85 |
| Immunohistochemistry, % | ||||
| CD45 | 100 | Not reported | Positive | Negative |
| CD30 | 70-85 (weak) | 31 (weak) | ∼85-100 (strong) | 100 |
| CD15 | Negative | Negative | 58-80 | 75-85 |
| CD20 CD79a PAX-5 MUM1 | 100 100 100 75 | 100 Not reported* Not reported 19 | ∼72-98 (weak) 67-75 98 Positive | Very rare (minority) Very rare 95 (weak) 100 |
| MAL CD23 | 60-70 85 | 57 71 | 30-49* 30-49 | 19 Negative |
| TRAF1 expression Nuclear cRel | 60-70 60-70 | Not reported Not reported | Usually positive Not reported | 84 92 |
| PDL1 PDL2 | 71 72 | 19 14 | ∼80 ∼30 | 87 |
| Genetic aberrations, % | ||||
| CIITA rearrangements 9p.24.1 aberrations (JAK2, PDL) 2p16.1 (Rel, BCL11A) SOCS1 mutation/deletion B2M mutations | 38 70 25-50 35-45 30-64 | Negative 33 Frequent 100 ∼15 | 30-37 60 33 40 32 | 15 25-30 25 42 40 |
MGZL, B-cell lymphoma unclassifiable with features intermediate between diffuse large B-cell lymphoma and classic Hodgkin lymphoma. Estimates for MGZL (vs non-MGZL) are reported where possible.12,20,21,23,42,90-92 Frequencies are provided when available; immunohistochemistry frequencies may vary depending on the thresholds used. For GZL, frequencies vary for cHL-like and large B-cell lymphoma subtypes (eg, MAL and CD23 expression are higher in large B-cell lymphoma type GZL).
NScHL, nodular sclerosing cHL.
As a subtype of DLBCL, CD79a, and PAX5 would be expected to show frequencies of 100%. The survival estimate is based on only 16 cases and requires validation.