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 |
PUCS papulovesicles, ulcers, crust, scars, HMB Hypersensitivity to mosquito bites, FHSL fever, hepatosplenomegaly, lymphadnopathy, FHS fever, hepatosplenomegaly, FL fever, lymphadnopathy, NP nodules nasopharynx nodules, HPS hemophagocytic syndrome, LN lymph node, BM bone marrow, BM- BM not involved, AWD Alive with disease, DOD dead of disease, NA not applicable, ND not done, LFU lost to follow-up, IMR interferon, mechlorethamine, and retinoic acid, NHL Non Hodgkin lymphoma, BFM Berlin-Frankfurt-Münster, MTX methotrexate, VP-16 etoposide, P-ASP pegaspargase, HSCT hematopoietic stem cell transplantation, GEM gemcitabine, L-OHP oxaliplatin, VCR vincristine, VLB vinblastine, PDN prednisone, CR complete remission, PD progressive disease, PR partial remission