Clinical features of HVLPD patients
| Patient . | Race/ sex . | Age at onset . | Age at last follow-up . | EBV-related symptoms . | Classification of HV . | Additional clinical features . | Treatment . |
|---|---|---|---|---|---|---|---|
| 1 | WM | 3 | 24 | None | Classic | None | None |
| 2 | WF | 5 | 15 | None | Classic | None | None |
| 3 | WM | 4 | 12 | None | Classic | Urticaria pigmentosa | Hydroxychloroquine |
| 4 | WM | 7 | 19 | None | Classic | Tetralogy of Fallot | None |
| 5 | WM | 6 | 28 | GI LPD | Systemic | Moebius syndrome | Hydroxychloroquine |
| 6 | WF | 3 | 16 | None | Classic | None | None |
| 7 | WF | 5 | 12 | None | Classic | Branchial cleft cyst on neck | Topical steroid |
| 8 | WF | 7 | 19 | None | Classic | Severe adenovirus conjunctivitis | Hydroxychloroquine, valacyclovir, prednisone pulses |
| 9 | WF | 7 | 16 | None | Classic | None | Prednisone pulses, valacyclovir, TL-01 narrow band UV-B and UV-A desensitization |
| 10 | WF | 3 | 11 | None | Classic | None | Valacyclovir, TL-01 (narrow-band UV-B) desensitization |
| 11 | HM | 3 | 16 | Hepatitis, lymphadenopathy, fever | Systemic | None | HSCT from 5/6 matched related donor |
| 12 | HM | 39 | 47 | None | Classic | None | None |
| 13 | AF | 10 | 27 | GI disease, pleural, muscle disease; T cell lymphoma of lung | Systemic | GATA2 deficiency | Prednisone, etoposide, chemotherapy, haploidentical HSCT from sister |
| 14 | AM | 8 | 23 | Hepatitis, fever, fatigue; NK/T-cell lymphoma after HSCT | Systemic | Renal insufficiency | Anakinra, prednisone, haploidentical HSCT from sister |
| 15 | HF | 6 | 15 | Fever, delirium, lymphocytic meningitis* | Systemic | None | Ganciclovir, intrathecal methotrexate |
| 16 | HM | 6 | 13 | Fever, abdominal pain | Systemic | None | Hydroxychloroquine, thalidomide, valacyclovir, HSCT |
| Patient . | Race/ sex . | Age at onset . | Age at last follow-up . | EBV-related symptoms . | Classification of HV . | Additional clinical features . | Treatment . |
|---|---|---|---|---|---|---|---|
| 1 | WM | 3 | 24 | None | Classic | None | None |
| 2 | WF | 5 | 15 | None | Classic | None | None |
| 3 | WM | 4 | 12 | None | Classic | Urticaria pigmentosa | Hydroxychloroquine |
| 4 | WM | 7 | 19 | None | Classic | Tetralogy of Fallot | None |
| 5 | WM | 6 | 28 | GI LPD | Systemic | Moebius syndrome | Hydroxychloroquine |
| 6 | WF | 3 | 16 | None | Classic | None | None |
| 7 | WF | 5 | 12 | None | Classic | Branchial cleft cyst on neck | Topical steroid |
| 8 | WF | 7 | 19 | None | Classic | Severe adenovirus conjunctivitis | Hydroxychloroquine, valacyclovir, prednisone pulses |
| 9 | WF | 7 | 16 | None | Classic | None | Prednisone pulses, valacyclovir, TL-01 narrow band UV-B and UV-A desensitization |
| 10 | WF | 3 | 11 | None | Classic | None | Valacyclovir, TL-01 (narrow-band UV-B) desensitization |
| 11 | HM | 3 | 16 | Hepatitis, lymphadenopathy, fever | Systemic | None | HSCT from 5/6 matched related donor |
| 12 | HM | 39 | 47 | None | Classic | None | None |
| 13 | AF | 10 | 27 | GI disease, pleural, muscle disease; T cell lymphoma of lung | Systemic | GATA2 deficiency | Prednisone, etoposide, chemotherapy, haploidentical HSCT from sister |
| 14 | AM | 8 | 23 | Hepatitis, fever, fatigue; NK/T-cell lymphoma after HSCT | Systemic | Renal insufficiency | Anakinra, prednisone, haploidentical HSCT from sister |
| 15 | HF | 6 | 15 | Fever, delirium, lymphocytic meningitis* | Systemic | None | Ganciclovir, intrathecal methotrexate |
| 16 | HM | 6 | 13 | Fever, abdominal pain | Systemic | None | Hydroxychloroquine, thalidomide, valacyclovir, HSCT |
A, Asian; F, female; GI, gastrointestinal; H, Hispanic; LPD, lymphoproliferative disease; M, male; W, white.
This patient developed systemic disease long after the onset of HV (9 years) manifested by lymphocytic meningitis; unlike the other patients with systemic disease, this patient has indeterminate T-cell clonality, which may explain the indolent course of her disease.