UCD and MCD case demographics
| Case . | Subtype . | Sex . | Age, y . | Site of involvement/ lymphadenopathy . | Clinical sequelae . | Organomegaly . | Viral status . | Laboratory results . | Treatment . | Status (time of follow-up) . |
|---|---|---|---|---|---|---|---|---|---|---|
| 1 | UCD-HVV | M | 26 | Interaortocaval right lower quadrant | Isolated adenopathy | None | HIV−, HHV8− | NS | Resection | Alive (10 y) |
| 2 | UCD-HVV | M | 35 | Head/right neck | Isolated adenopathy | None | HHV8− | NS | Resection | Alive (2 y) |
| 3 | iMCD-PC (2 major criteria; 8 minor criteria) | F | 70 | Axillary, inguinal, mediastinal, intraabdominal lymphadenopathy | Fatigue, edema and pleural effusions; TAFRO syndrome | None | HHV8−, HIV−, EBV− | ESR, 98 mm/h; CRP, 11.16 mg/L; Hgb, 8.1 g/dL; PLT, 49 × 109/L; IgG 2230 mg/dL; albumin, 2.6 g/dL; eGFR 36 mL/min/m2 | Resection, Rituximab-Etoposide/Siltuximab | Alive (2 y) |
| 4 | UCD-HVV | F | 40 | Left axillary and subpectoral lymphadenopathy | Localized adenopathy | None | HHV8−, HIV−, EBV− | CRP, 3.1 mg/dL; Hgb, 9.4 g/dL | Resection | Alive (4 y) |
| 5 | UCD-MV | F | 59 | Bilateral cervical lymphadenopathy/nasopharyngeal mass | Extensive multinodal adenopathy | None | HHV8−, HIV−, EBV− | Resection, tocilizumab | Alive (4 y) | |
| 6 | UCD-PCV | F | 57 | Left neck lymphadenopathy | Extensive localized adenopathy | None | HHV8−, EBV− | CRP, 2.7 mg/dL | Resection, rituximab | Alive (5 y) |
| 7 | UCD-HVV | F | 28 | Left neck lymphadenopathy | Localized adenopathy | None | HIV−, HHV8− | NS | Resection | Alive (5 y) |
| 8 | iMCD-PC (2 major criteria; 4 minor criteria) | M | 35 | Large anterior mediastinal mass, cervical lymphadenopathy | Weight loss, night sweats, fatigue | None | HHV8−, HIV−, EBV− | Hgb, 12.1 g/dL; PLT, 568 × 109/L; Hypergamma-globulinemia | Resection | Alive (3 y) |
| 9 | UCD-HVV | M | 30 | Retroperitoneal lymphadenopathy | Isolated adenopathy | None | HHV8− | NS | Resection | Alive (6 y) |
| 10 | UCD-HVV | F | 42 | Left pelvic lymphadenopathy | Isolated adenopathy | None | HHV8−, HIV− | NS | Resection, XRT | Alive (4 y) |
| 11 | iMCD-M (2 major; 7 minor criteria) | M | 21 | Diffuse retroperitoneal, inguinal lymphadenopathy | TAFRO syndrome; fatigue, fever, weight loss; hepatomegaly; edema | Hepatospleno-megaly | HHV8−, EBV−, HIV− | CRP, 16.4 mg/dL; Hgb, 7.9 g/dL; PLT, 77 K/μL; eGFR, 43 mL/min/m2 | Siltuximab, rituximab | Alive (2 y) |
| 12 | UCD-HVV | F | 17 | Neck lymphadenopathy | Localized adenopathy | N/A | HHV8− | NS | Resection, localized XRT | Alive (11 y) |
| 13 | UCD-HVV | M | 30 | Anterior mediastinal mass | Localized mass | N/A | HHV8− | NS | Resection, steroids | Alive (4 y) |
| 14 | UCD-HVV | M | 36 | Neck lymphadenopathy | Localized mass | None | HHV8− | NS | Resection, XRT | Alive (1 y) |
| 15 | UCD-HVV | F | 17 | Left adnexal mass | Localized mass | None | HHV8− | NS | Resection | Alive (4 y) |
| 16 | UCD-HVV | F | 24 | Anterior mediastinal mass | Fatigue; localized mass | N/A | HHV8− | NS | Resection | Alive (13 y) |
| 17 | UCD-HVV | F | 14 | Paratracheal mediastinal mass | Localized mass | N/A | HHV8− | NS | Resection | Alive (8 y) |
| 18 | UCD-HVV | F | 29 | Pelvic mass | Isolated adenopathy | None | HHV8− | NS | Resection | Alive (2 y) |
| Case . | Subtype . | Sex . | Age, y . | Site of involvement/ lymphadenopathy . | Clinical sequelae . | Organomegaly . | Viral status . | Laboratory results . | Treatment . | Status (time of follow-up) . |
|---|---|---|---|---|---|---|---|---|---|---|
| 1 | UCD-HVV | M | 26 | Interaortocaval right lower quadrant | Isolated adenopathy | None | HIV−, HHV8− | NS | Resection | Alive (10 y) |
| 2 | UCD-HVV | M | 35 | Head/right neck | Isolated adenopathy | None | HHV8− | NS | Resection | Alive (2 y) |
| 3 | iMCD-PC (2 major criteria; 8 minor criteria) | F | 70 | Axillary, inguinal, mediastinal, intraabdominal lymphadenopathy | Fatigue, edema and pleural effusions; TAFRO syndrome | None | HHV8−, HIV−, EBV− | ESR, 98 mm/h; CRP, 11.16 mg/L; Hgb, 8.1 g/dL; PLT, 49 × 109/L; IgG 2230 mg/dL; albumin, 2.6 g/dL; eGFR 36 mL/min/m2 | Resection, Rituximab-Etoposide/Siltuximab | Alive (2 y) |
| 4 | UCD-HVV | F | 40 | Left axillary and subpectoral lymphadenopathy | Localized adenopathy | None | HHV8−, HIV−, EBV− | CRP, 3.1 mg/dL; Hgb, 9.4 g/dL | Resection | Alive (4 y) |
| 5 | UCD-MV | F | 59 | Bilateral cervical lymphadenopathy/nasopharyngeal mass | Extensive multinodal adenopathy | None | HHV8−, HIV−, EBV− | Resection, tocilizumab | Alive (4 y) | |
| 6 | UCD-PCV | F | 57 | Left neck lymphadenopathy | Extensive localized adenopathy | None | HHV8−, EBV− | CRP, 2.7 mg/dL | Resection, rituximab | Alive (5 y) |
| 7 | UCD-HVV | F | 28 | Left neck lymphadenopathy | Localized adenopathy | None | HIV−, HHV8− | NS | Resection | Alive (5 y) |
| 8 | iMCD-PC (2 major criteria; 4 minor criteria) | M | 35 | Large anterior mediastinal mass, cervical lymphadenopathy | Weight loss, night sweats, fatigue | None | HHV8−, HIV−, EBV− | Hgb, 12.1 g/dL; PLT, 568 × 109/L; Hypergamma-globulinemia | Resection | Alive (3 y) |
| 9 | UCD-HVV | M | 30 | Retroperitoneal lymphadenopathy | Isolated adenopathy | None | HHV8− | NS | Resection | Alive (6 y) |
| 10 | UCD-HVV | F | 42 | Left pelvic lymphadenopathy | Isolated adenopathy | None | HHV8−, HIV− | NS | Resection, XRT | Alive (4 y) |
| 11 | iMCD-M (2 major; 7 minor criteria) | M | 21 | Diffuse retroperitoneal, inguinal lymphadenopathy | TAFRO syndrome; fatigue, fever, weight loss; hepatomegaly; edema | Hepatospleno-megaly | HHV8−, EBV−, HIV− | CRP, 16.4 mg/dL; Hgb, 7.9 g/dL; PLT, 77 K/μL; eGFR, 43 mL/min/m2 | Siltuximab, rituximab | Alive (2 y) |
| 12 | UCD-HVV | F | 17 | Neck lymphadenopathy | Localized adenopathy | N/A | HHV8− | NS | Resection, localized XRT | Alive (11 y) |
| 13 | UCD-HVV | M | 30 | Anterior mediastinal mass | Localized mass | N/A | HHV8− | NS | Resection, steroids | Alive (4 y) |
| 14 | UCD-HVV | M | 36 | Neck lymphadenopathy | Localized mass | None | HHV8− | NS | Resection, XRT | Alive (1 y) |
| 15 | UCD-HVV | F | 17 | Left adnexal mass | Localized mass | None | HHV8− | NS | Resection | Alive (4 y) |
| 16 | UCD-HVV | F | 24 | Anterior mediastinal mass | Fatigue; localized mass | N/A | HHV8− | NS | Resection | Alive (13 y) |
| 17 | UCD-HVV | F | 14 | Paratracheal mediastinal mass | Localized mass | N/A | HHV8− | NS | Resection | Alive (8 y) |
| 18 | UCD-HVV | F | 29 | Pelvic mass | Isolated adenopathy | None | HHV8− | NS | Resection | Alive (2 y) |
CRP, C-reactive protein; EBV, Epstein-Barr virus; eGFR, estimated glomerular filtration rate; ESR, erythrocyte sedimentation rate; F, female; Hgb, hemoglobin; IgG, immunoglobulin G; iMCD-M, HHV8−/iMCD with mixed pathology; iMCD-PC, HHV8−/iMCD with plasmacytic pathology; M, male; N/A, not available; NS, not significant; PLT, platelet; XRT, radiotherapy.