Table 1 Clinicopathologic features of patients with HV-LPD
Case | Age(y)/ Sex/History (y) | Skin lesions/location | Systemic manifestations | Size of atypical cells/infiltration depth/necrosis | LN, BM biopsy | LDH (IU/L) | Phenotype | Therapy | Best response to treatment | Current status | Survival after Diagnosis |
|---|---|---|---|---|---|---|---|---|---|---|---|
1 | 9/F/1 | PUCS/face, scalp, extremities | FHSL | Medium/dermis | EBV+ cells in interfollicular area of LN | 324 | CD8 | Chemotherapy (IMR) and Radiotherapy. Maintained herbal remedy | CR | AWD | 8.7Y |
2 | 13/F/3 | Erythema, PUCS/face, extremities, and areas not exposed to sun | FHSL | Medium/dermis and fatty tissue/Fatty tissue necrosis | EBV+ cells diffusely infiltrated LN | 624 | CD56 | Chemotherapy (NHL-BFM-90) for 3 cycles [14] | PD | DOD | 1.1Y |
3 | 8/M/1 | PUCS, nodules/face, extremities | FHSL | Medium/dermis | EBV+ cells present in interfollicular area of LN | 450 | CD56 | Chemotherapy (MTX + VP-16) and Radiotherapy | PR | AWD | 7.6Y |
4 | 4/M/1 | PUCS/ areas exposed to sun; HMB | Fever | Small and medium/dermis and subcutaneous tissue | ND | NA | CD4 | Herbal remedy | PD | AWD | 6Y |
5 | 5/M/1 | PUCS, nodules/face, extremities | FHS | Small and medium/dermis | ND | NA | CD8 | Chemotherapy (IMR) and Radiotherapy. Maintained Herbal remedy | PD | DOD | 4.4Y |
6 | 8/F/1.5 | PUCS/face, arms, areas not exposed to sun | FHSL | Medium and large/dermis | ND | NA | CD56 | NA | NA | DOD | 3Y |
7 | 10/M/3 | PUCS/face, extremities, HMB | FL | Small and medium/dermis and subcutaneous tissue | ND | 298.4 | CD8 | Chemotherapy (NHL-BFM-95) for 6 cycles [15] | PR | DOD | 2.2Y |
8 | 2/F/0.5 | PUCS, nodules/face, extremities | None | Medium/dermis and subcutaneous tissue | ND | NA | CD56 | NA | NA | LFU | LFU |
9 | 21/M/14 | PUCS/areas exposed and unexposed to sun; HMB | FSHLNP nodules, HPS | Medium and large/dermis and subcutaneous tissue | EBV+ cells diffusely infiltrated LN and BM | 1202 | CD4 | Chemotherapy (GEM+VP16 + MTX + P-ASP+thalidomide for 6 cycles) | PR | DOD | 0.6Y |
10 | 7/F/1 | PUCS/face, extremities, | Fever | Medium/dermis and fatty tissue | ND | NA | CD8 | NA | NA | LFU | LFU |
11 | 5/M/2 | PUCS, nodules/face, extremities, trunk; HMB | FHSL | Medium/dermis | EBV+ cells in interfollicular area of LN | 318 | CD4 | Chemotherapy (NHL-BFM-95) for 6 cycles; Sibling HSCT; and EBV-CTL treatment [15] | CR | Alive without disease | 4.4Y |
12 | 13/F/1 | PUCS/face, extremities, trunk, and oral cavity | FL | Medium/dermis | BM- | 482 | CD56 | Chemotherapy (P-ASP+GEM+L-OHP) Maintained herbal remedy | PD | DOD | 2.2Y |
13 | 12/F/2 | PUCS, nodules/face, trunk, and extremities | FHSL | Medium/dermis and fatty tissue | EBV+ cells diffusely infiltrated LN and BM | 358.9 | CD56 | Chemotherapy (GEM for 6 cycles) and antivirus treatment (Ganciclovir) | CR | AWD | 3.2Y |
14 | 5/M/3 | PUCS/face, extremities, trunk | FL | Medium/dermis and subcutaneous tissue | BM- | 334.6 | CD4 | Chemotherapy (First Line: GEM for 6 cycles, Second Line: VP-16 + VCR for 1 cycle, Third Line: VLB + PDN for 1 cycle) | PR | AWD | 2.9Y |
15 | 7/F/4 | PUCS/face, extremities | FL | Medium/dermis | BM- | 323.2 | CD56 | Chemotherapy (GEM for 6 cycles) | CR | DOD | 1.3Y |
16 | 12/F/4 | PUCS/face, trunk, arms and legs; HMB | FHSL | Medium/dermis and fatty tissue | BM- | NA | CD4 | NA | NA | DOD | 0.7Y |
17 | 11/M/9 | PUCS, nodules/face, arms, legs, and areas not exposed to sun; HMB | FHSL | Medium/dermis and subcutaneous tissue | EBV+ cells diffusely infiltrated LN and BM | 585 | CD4 | Chemotherapy (First Line: NHL-BFM-95, Second Line: GEM for 2 cycles) [15] | PD | DOD | 2.4Y |
18 | 8/M/2 | PUCS/face, arms, legs, and trunk | Fever | Medium/dermis and subcutaneous tissue | BM- | 312 | CD4 | Chemotherapy (methylprednisolone, azithromycin, acitretin, and cyclosporine) | PR | AWD | 2.2Y |
19 | F/5/3 | PUCS/face, legs, and trunk | Fever | Medium/dermis and subcutaneous tissue | ND | NA | CD8 | NA | NA | LFU | LFU |