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Table 1 Clinicopathologic features of patients with HV-LPD

From: Clinicopathological categorization of hydroa vacciniforme-like lymphoproliferative disorder: an analysis of prognostic implications and treatment based on 19 cases

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
  1. 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
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