RESUMEN

Purpose: To examine the clinical and pathological features, laboratory markers, therapeutic options and risk factors indicating poor prognosis of hydroa vacciniforme-like lymphoproliferative disorder (HVLPD). Patients and Methods: Seven patients with HVLPD had their clinical and pathological data collected. Immunohistochemical staining, Epstein-Barr virus-encoded RNA (EBER) in situ hybridization experiments, T-cell receptor (TCR) gene rearrangement, RT-PCR tests and the Elisa assay were carried out. Results: The main clinical manifestations were papulovesicular lesions and ulcers on the face, neck, or trunk. Five cases had systemic symptoms. Three of the deceased patients had significant facial edema, deep body necrosis, and ulceration. The pathological results demonstrated that lymphocytes infiltrated blood vessels and sweat glands in addition to the dermis and subcutaneous tissues. All patients tested positive for CD3 and EBER. Six cases tested positive for TCRßF1, but none tested positive for TCRδ. TCRγ monoclonal rearrangement, strongly positive expression of TIA-1 and a Ki67 proliferation index of 40% occurred in 3 fatal cases. When compared to the survival group, the plasma EBV DNA in the deceased group was considerably higher (P<0.05). IFN-γ and TNF-α cytokine levels in patients were higher than in the control group, particularly in the deceased group (P<0.05). The skin lesions on all patients recovered quickly underwent conservative care. Nonetheless, 3 patients passed away as the disease progressed in its latter stages. Conclusion: In our cases, the main infiltrating cells were T cells and the dominant lymphocyte subclass was αßT cells. A significant increase in lgE level, plasma EBV DNA, IFN-γ, and TNF-α cytokine levels, decreased hemoglobin level, strongly positive expression of TIA-1, high Ki67 proliferation index, and positive TCR gene rearrangement are all indicators of a poor prognosis.

RESUMEN

The clinicopathological features of 32 patients (17 females and 15 males) with a median age of 8 years (range, 1.5-21 years) from Southwestern China diagnosed with hydroa vacciniforme-like lymphoproliferative disorder (HVLPD) were reviewed. At presentation, 6 patients showed only skin lesions, while 26 patients showed both skin lesions and systemic symptoms, including fever, lymphadenopathy and hepatosplenomegaly. As the disease progressed, systemic symptoms occurred in all patients. Follow-up data of 29 patients showed that 14 patients were still alive with disease with a median follow-up time of 22 months (range 3.6-71 months), and 15 patients died within a median follow-up of 6 months (range 0-60 months).

Asunto(s)

Infecciones por Virus de Epstein-Barr , Hidroa Vacciniforme , Trastornos Linfoproliferativos , Adolescente , Adulto , Niño , Preescolar , Infecciones por Virus de Epstein-Barr/complicaciones , Infecciones por Virus de Epstein-Barr/diagnóstico , Infecciones por Virus de Epstein-Barr/patología , Femenino , Herpesvirus Humano 4 , Humanos , Hidroa Vacciniforme/diagnóstico , Hidroa Vacciniforme/patología , Lactante , Trastornos Linfoproliferativos/diagnóstico , Trastornos Linfoproliferativos/patología , Masculino , Estudios Retrospectivos , Adulto Joven

RESUMEN

PURPOSE: To report a case of ocular involvement associated with hydroa vacciniforme-like lymphoproliferative disorder (HVLPD). CASE REPORT: A 10-year-old HVLPD boy suffered conjunctivitis, interstitial keratitis, and anterior uveitis sequentially during the whole course. Interestingly, this case manifested initially only with ocular findings, which preceded 1 year earlier than the onset of cutaneous lesions. And his later ocular findings occurred simultaneously with cutaneous lesions. The patient was treated with oral prednisone, ganciclovir, and light protection. Topical corticosteroid drops used to control ocular inflammation. Since then, he has not had any flares of ocular inflammation, and the cutaneous lesions improved. Although corneal nebula had been formed, the vision was still good. CONCLUSION: Our case was supportive of ocular involvement in HVLPD. Ophthalmologists should be aware of ocular involvement in HVLPD could be preceded the onset of cutaneous lesions, and prudently perform a careful ophthalmic examination at regular intervals to limit long-term sequelae.

Asunto(s)

Infecciones por Virus de Epstein-Barr , Hidroa Vacciniforme , Queratitis , Trastornos Linfoproliferativos , Neoplasias Cutáneas , Niño , Infecciones por Virus de Epstein-Barr/complicaciones , Infecciones por Virus de Epstein-Barr/diagnóstico , Infecciones por Virus de Epstein-Barr/tratamiento farmacológico , Humanos , Hidroa Vacciniforme/complicaciones , Hidroa Vacciniforme/diagnóstico , Hidroa Vacciniforme/tratamiento farmacológico , Inflamación/complicaciones , Queratitis/etiología , Trastornos Linfoproliferativos/complicaciones , Trastornos Linfoproliferativos/diagnóstico , Trastornos Linfoproliferativos/tratamiento farmacológico , Masculino , Neoplasias Cutáneas/complicaciones

RESUMEN

Hydroa vacciniforme-like lymphoproliferative disorder (HVLPD) is characterized by vesiculopapular eruptions on sun-exposed and sometimes unexposed skin. Though ocular involvement in HVLPD is rare, it may present with conjunctivitis, corneal opacities, uveitis, and interstitial keratitis. We report a case of a 7-year-old boy with HVLPD, whose ophthalmic symptoms were neglected for over 2 years, who developed anterior uveitis and corneal nebulae without vision impairment. Awareness of eye involvement in patients with HVLPD may help to prevent severe complications.

Asunto(s)

Infecciones por Virus de Epstein-Barr , Hidroa Vacciniforme , Queratitis , Trastornos Linfoproliferativos , Niño , Humanos , Hidroa Vacciniforme/diagnóstico , Trastornos Linfoproliferativos/diagnóstico , Masculino , Piel

RESUMEN

BACKGROUND: Hydroa vacciniforme-like lymphoproliferative disorder (HVLPD) is a chronic Epstein-Barr virus (EBV)-positive lymphoproliferative disease which may either present as an indolent condition or progress to a systemic T-cell lymphoma. METHODS: All HVLPD diagnosed over a 10-year period were retrieved, and clinical data regarding sex, age, oral and systemic manifestations, and clinical follow-up were obtained. Immunohistochemistry was done in order to characterize the lymphoid cells, and in situ hybridization was used to demonstrate the presence of EBV. RESULTS: Eleven cases were included, with a male predominance and a mean age of 25.1 years. Buccal mucosa and the lips were the most affected oral sites, appearing as painful ulcers. All patients exhibited facial oedema, usually affecting the lips, nose and periorbital region. The clinical course was gradual but progressive, with four patients having fever and 3 showing lymphadenopathies. All cases showed a moderate to severe lymphocytic infiltrate with angiotropism, angiocentricity and epidermotropism. Two cases affecting the lip skin exhibited a periappendageal lymphocytic infiltrate. Few large pleomorphic cells were found, surrounded by smaller and medium-sized lymphoid cells, as well as reactive plasma cells, macrophages, neutrophils and eosinophils. All lesions exhibited a cytotoxic T-cell (CD8+) phenotype with a variable proliferative index. All cases were associated with EBV, and all patients died due to complications of the disease. CONCLUSIONS: HVLPD is a rare disease that may show oral involvement with a cytotoxic T-cell phenotype, and is strongly associated with EBV. As shown in this series, HVLPD may show aggressive clinical behaviour.

Asunto(s)

Infecciones por Virus de Epstein-Barr , Hidroa Vacciniforme , Trastornos Linfoproliferativos , Adulto , Infecciones por Virus de Epstein-Barr/complicaciones , Femenino , Herpesvirus Humano 4 , Humanos , Masculino , Perú

RESUMEN

Primary cutaneous T-cell lymphomas pose a diagnostic challenge for dermatopathologists, hematopathologists, and general surgical pathologists. Recognition of gamma/delta phenotype in cutaneous T proliferations has been enhanced by the availability of antibodies against TCRgamma and delta for immunohistochemistry. Thus, reporting gamma/delta phenotype in a cutaneous T-cell lymphoid proliferation may indicate a significant change in therapy and a challenge for dermatologists and oncologists who treat these patients. Herein, we discuss primary cutaneous gamma/delta T-cell lymphoma, its differential diagnosis, and other skin lymphoid proliferations that may show gamma/delta phenotype. Awareness of the occurrence of gamma/delta phenotype in both T-cell lymphomas and benign lymphoid proliferations involving skin is crucial for a better interpretation of histopathologic findings. Integration of clinical presentation, morphology, immunoprofile, and molecular findings is key for a correct diagnosis and appropriate therapy of lesions displaying gamma/delta T-cell phenotype.

Asunto(s)

Linfoma Cutáneo de Células T , Neoplasias Cutáneas , Humanos , Linfoma Cutáneo de Células T/diagnóstico , Linfoma Cutáneo de Células T/genética , Fenotipo , Receptores de Antígenos de Linfocitos T gamma-delta/genética , Neoplasias Cutáneas/diagnóstico , Neoplasias Cutáneas/genética , Linfocitos T

RESUMEN

Hydroa vacciniforme-like lymphoproliferative disorder (HV-LPD) is a rare cutaneous disease associated with Epstein-Barr virus infection. We retrospectively analyzed the clinical presentation, histopathological characteristics, and prognostic study of HV-LPD in 24 Chinese patients. All patients presented with recurrent papulovesicular and necrotic eruptions on the face, neck, and extremities, with 11 showing systemic symptoms. Twenty patients were diagnosed with HV-LPD in childhood (age < 18 years) and four in adulthood (age ≥ 18 years). The median age at diagnosis was 8.5 years old (range, 2-50). Histopathology revealed variably dense lymphocyte infiltration throughout the dermis. All cases were strongly positive for CD3 and Epstein-Barr encoding region based on in situ hybridization. Of 18 cases with a T-cell phenotype, 15 harbored monoclonal rearrangements in T-cell receptor (TCR) genes. Four cases with a natural killer cell phenotype carried polyclonal rearrangements in TCR genes. Among 24 patients, eight (33.3%) received chemotherapy, two (8.3%) allogeneic hematopoietic stem cell transplantation, and both are currently alive without disease. The median follow-up period was 24 months (range, 7-120) and 23 patients were available: 15 (62.5%) were alive, and eight (33.3%) had died. Fourteen cases had a relapse of disease and three developed lymphoma within 24 months of diagnosis. The mean survival time of childhood-onset patients was longer than that of adult-onset patients (36.4 vs. 20.8 months). In summary, the wide clinical course and representative presentation of cases in our center reflect the pedigree characteristics of HV-LPD. Allogeneic hematopoietic stem cell transplantation should be a preferred choice for relapse and refractory patients due to the poor effect of chemotherapy. Adult-onset and high serum EBV DNA loads may indicate an increased risk of aggressive disease in patients with HV-LPD.

Asunto(s)

Infecciones por Virus de Epstein-Barr , Hidroa Vacciniforme , Linfoma , Trastornos Linfoproliferativos , Adolescente , Adulto , Niño , China/epidemiología , Infecciones por Virus de Epstein-Barr/diagnóstico , Infecciones por Virus de Epstein-Barr/epidemiología , Herpesvirus Humano 4/genética , Humanos , Hidroa Vacciniforme/diagnóstico , Hidroa Vacciniforme/epidemiología , Hidroa Vacciniforme/terapia , Trastornos Linfoproliferativos/diagnóstico , Trastornos Linfoproliferativos/terapia , Recurrencia Local de Neoplasia , Pronóstico , Estudios Retrospectivos

RESUMEN

BACKGROUND: Hydroa Vacciniforme-like Lymphoproliferative Disorder (HV-LPD) is the name given to a group of Epstein-Barr virus (EBV)-associated diseases. It resembles hydroa vacciniforme (HV), the rarest form of photosensitivity, and is a T-cell disorder associated with an Epstein-Barr virus infection. The majority of diagnosed cases occur in East Asia and South America. It is rare in the United States and Europe. Multiple studies have revealed the clinical manifestation of an enlarged liver, but no gold standard such as pathology has yet supported this as a clinical sign of HV-LPD. CASE PRESENTATION: Here, we report a case of a 34-year-old Asian female with definite liver invasion. The patient had complained of a recurring facial rash for many years. The patient was admitted to the hospital because of an enlarged liver. After hospitalization, she was given an EB virus nucleic acid test. The EB virus nucleic acid test was positive, and pathological examination suggested that HV-LPD had invaded the skin, bone marrow, and liver. After being given antiviral treatment, the patient's symptoms were mitigated. CONCLUSIONS: Our case confirms the liver damage was caused by HV-LPD and the effectiveness of antiviral treatment.

Asunto(s)

Médula Ósea/patología , Infecciones por Virus de Epstein-Barr/complicaciones , Infecciones por Virus de Epstein-Barr/diagnóstico , Herpesvirus Humano 4/genética , Hidroa Vacciniforme/complicaciones , Hidroa Vacciniforme/diagnóstico , Hígado/patología , Trastornos Linfoproliferativos/complicaciones , Trastornos Linfoproliferativos/diagnóstico , Adulto , Antivirales/uso terapéutico , Beijing , Médula Ósea/virología , Infecciones por Virus de Epstein-Barr/tratamiento farmacológico , Infecciones por Virus de Epstein-Barr/virología , Exantema/complicaciones , Exantema/tratamiento farmacológico , Femenino , Hepatomegalia/tratamiento farmacológico , Hepatomegalia/virología , Humanos , Hidroa Vacciniforme/tratamiento farmacológico , Hidroa Vacciniforme/patología , Hígado/virología , Linfoma de Células T/complicaciones , Linfoma de Células T/tratamiento farmacológico , Linfoma de Células T/virología , Trastornos Linfoproliferativos/tratamiento farmacológico , Trastornos Linfoproliferativos/patología , Piel/patología , Resultado del Tratamiento

RESUMEN

BACKGROUND: Hydroa vacciniforme-like lymphoproliferative disorder (HVLPD) encompasses a rare group of Epstein-Barr virus (EBV)-associated lymphoproliferative diseases. OBJECTIVE: To define the clinical and pathologic characteristics of HVLPD and to identify mutant genes that may be related to the development of HVLPD. METHODS: Clinical data and archived formalin-fixed, paraffin-embedded tissue were obtained from 19 patients. Specimens were analyzed by immunohistochemistry and in situ hybridization to detect EBV-encoded RNA (EBER1/2) and for T cell receptor (TCR) gene rearrangements. Whole-exome sequencing (WES) analysis was also performed in this study. RESULTS: Thirteen patients survived between 3-58 months (median, 21 months) during the follow-up. Six patients who were almost adults (>15 years old) and died of the disease presented with facial edema. Lactate dehydrogenase (LDH) levels were elevated, and the TCR gene rearrangement test was positive more frequently in the patients who died. Compared with Chinese patients in a similar previous report, our patients had significantly higher proliferation (in all cases, the Ki-67 index was greater than 10 %) and a more aggressive clinical course. Moreover, after WES and Sanger verification, STAT3, IKBKB, ELF3, CHD7, KMT2D, ELK1, RARB and HPGDS were screened out in our patients. CONCLUSIONS: HVLPD refers to a heterogeneous group of cutaneous lymphoproliferative diseases with different clinical and pathological features that affect patient outcomes. Gene mutations may be correlated with the development of HVLPD, and our study may provide new therapeutic targets for HVLPD.

Asunto(s)

Infecciones por Virus de Epstein-Barr/diagnóstico , Herpesvirus Humano 4/aislamiento & purificación , Hidroa Vacciniforme/diagnóstico , Trastornos Linfoproliferativos/diagnóstico , Piel/patología , Adolescente , Adulto , Biomarcadores/análisis , Proliferación Celular/genética , Niño , Preescolar , China , Análisis Mutacional de ADN , Infecciones por Virus de Epstein-Barr/genética , Infecciones por Virus de Epstein-Barr/patología , Infecciones por Virus de Epstein-Barr/virología , Femenino , Estudios de Seguimiento , Herpesvirus Humano 4/genética , Humanos , Hidroa Vacciniforme/genética , Hidroa Vacciniforme/patología , Hidroa Vacciniforme/virología , Trastornos Linfoproliferativos/genética , Trastornos Linfoproliferativos/patología , Trastornos Linfoproliferativos/virología , Masculino , Persona de Mediana Edad , Mutación , Fotograbar , ARN Viral/aislamiento & purificación , Estudios Retrospectivos , Piel/diagnóstico por imagen , Piel/virología , Secuenciación del Exoma , Adulto Joven

RESUMEN

Epstein-Barr virus (EBV)-positive T-cell and natural killer (NK)-cell lymphoproliferative diseases (EBV-TNKLPD) are a group of uncommon disorders characterised by EBV infection of T- and NK-cells. As a group, EBV-TNKLPD are more commonly encountered in Asians and Native Americans from Central and South America compared to Western populations. They encompass a spectrum of entities that range from non-neoplastic lesions such as EBV-associated haemophagocytic lymphohistiocytosis (EBV-HLH) to more chronic conditions with variable outcomes such as chronic active EBV infections (CAEBV) of T- and NK-cell type (cutaneous and systemic forms) and malignant diseases such as systemic EBV-positive T-cell lymphoma of childhood, aggressive NK-cell leukaemia, extranodal NK/T-cell lymphoma, nasal-type, and primary EBV-positive nodal T/NK-cell lymphoma. Due to their rarity, broad clinicopathological spectrum and significant morphological and immunophenotypic overlap, the diagnosis and precise classification of EBV-TNKLPD often pose a challenge to clinicians and pathologists. Correct classification of this group of rare diseases relies heavily on the age of onset, disease presentation, duration of symptoms and cell of origin (T- vs NK-cell lineage). In this review, we provide an update on the clinicopathological and molecular features of the various EBV-TNKLPD entities occurring in non-immunocompromised patients and present a practical algorithmic approach for the general pathologist who is confronted with these disorders in routine clinical practice.

Asunto(s)

Infecciones por Virus de Epstein-Barr/virología , Herpesvirus Humano 4/patogenicidad , Células Asesinas Naturales/patología , Linfoma de Células T/patología , Trastornos Linfoproliferativos/patología , Infecciones por Virus de Epstein-Barr/diagnóstico , Humanos , Células Asesinas Naturales/virología , Leucemia Linfocítica Granular Grande/diagnóstico , Leucemia Linfocítica Granular Grande/patología , Leucemia Linfocítica Granular Grande/virología , Linfoma de Células T/virología , Trastornos Linfoproliferativos/diagnóstico , Trastornos Linfoproliferativos/virología

RESUMEN

BACKGROUND: Hydroa vacciniforme-like lymphoproliferative disorder (HV-LPD) is a cutaneous form of chronic active Epstein-Barr virus (EBV) infection, which occurs mainly in children in Latin America and Asia. It can progress to systemic lymphoma. However, prognostic factors and treatment remain unclear. METHODS: This retrospective study reviewed the clinical, morphologic, immunophenotypical features, and clinical treatment of 19 patients with HV-LPD. RESULTS: All 19 patients had skin lesions in the face, extremities, or areas unexposed to the sun, including edema, blistering, ulceration, and scarring. The course was slowly progressive and relapsing. Histopathology showed an atypical lymphocytic infiltrate in the dermis and/or subcutaneous tissue. The lesions had a cytotoxic T/NK-cell immunophenotype. Among 19 patients, 7 (37%) exhibited CD4+ T cells, 5 (26%) exhibited CD8+ T cells, and 7 (37%) exhibited CD56+ cells. Of 12 cases with a T-cell phenotype, molecular analyses demonstrated that 7 had monoclonal rearrangements in the T-cell receptor genes. Three cases had an NK-cell phenotype and had polyclonal rearrangements in the TCR genes. All cases were associated with EBV infections. Among 19 patients, 9 (47.4%) received chemotherapy. Only one patient received allogeneic transplantation and EBV-specific cytotoxic T lymphocyte treatment after chemotherapy. That patient was the only one alive without disease at the latest follow up. Nine patients died of systemic lymphoma with disease progression, indicating irreversible process. CONCLUSIONS: This study confirmed that HV-LPD is a broad-spectrum EBV+ lymphoproliferative disorder. It progressed to EBV+ systemic T/NK lymphoma, although some patients had a more indolent, chronic course. Cytopenia, elevated lactate dehydrogenase, destructive-multiorgan involvement, and older age were poor prognostic factors. Only allogeneic transplantation was curative.

Asunto(s)

Hidroa Vacciniforme/patología , Linfoma de Células T/patología , Trastornos Linfoproliferativos/patología , Recurrencia Local de Neoplasia/patología , Adolescente , Niño , Preescolar , Infecciones por Virus de Epstein-Barr/complicaciones , Femenino , Herpesvirus Humano 4/genética , Humanos , Hidroa Vacciniforme/diagnóstico , Hidroa Vacciniforme/virología , Células Asesinas Naturales/patología , Células Asesinas Naturales/virología , Linfoma de Células T/virología , Trastornos Linfoproliferativos/diagnóstico , Masculino , Pronóstico , Neoplasias Cutáneas/diagnóstico , Neoplasias Cutáneas/patología , Neoplasias Cutáneas/virología , Adulto Joven

RESUMEN

BACKGROUND: Hydroa vacciniforme-like lymphoproliferative disorder (HVLLPD) is a rare Epstein-Barr virus (EBV)-related disease that is usually found in East Asians and Latin Americans. OBJECTIVE: To report the characteristics of HVLLPD in Chinese patients. METHODS: Retrospective analysis of patients with HVLLPD from a single institute. RESULTS: A total of 41 patients were enrolled. All patients presented with papulovesicular lesions, mainly distributed on sun-exposed areas, with 26 patients showing systemic symptoms. Follow-up data were available for 20 patients, 16 patients were alive, and 4 patients died. Of the 4 deceased patients, 3 had taken a serum EBV DNA test that showed high viral loads. These 3 patients also received chemotherapy. Histopathology was characterized by dense proliferation of lymphocytes in the dermis. Angiotropism or angiodestruction was found in the majority of patients, whereas prominent cellular polymorphism was noticed in only 4 patients. All patients were positive for CD3, TIA1 cytotoxic granule associated RNA binding protein, and EBV-encoded RNA in situ hybridization. LIMITATIONS: This was a retrospective study. CONCLUSIONS: HVLLPD in Chinese patients showed indolent behavior in the majority of cases, which differed from the characteristics of HVLLPD in Latin Americans. Patients with high serum EBV DNA loads had an increased risk of their disease evolving into aggressive disease. Chemotherapy should not be considered as first-line treatment for most Chinese patients.

Asunto(s)

ADN Viral/sangre , Infecciones por Virus de Epstein-Barr/complicaciones , Herpesvirus Humano 4 , Trastornos Linfoproliferativos/sangre , Trastornos Linfoproliferativos/patología , Adolescente , Adulto , Antígenos CD/metabolismo , Relación CD4-CD8 , Niño , Preescolar , China , Infecciones por Virus de Epstein-Barr/sangre , Femenino , Humanos , Hidroa Vacciniforme/patología , Lactante , Linfocitos/metabolismo , Trastornos Linfoproliferativos/complicaciones , Trastornos Linfoproliferativos/virología , Masculino , Estudios Retrospectivos , Carga Viral , Adulto Joven

RESUMEN

Objective To analyze clinical features and prognosis of 15 children with hydroa vacciniforme-like lymphoproliferative disorder (HVLPD). Methods Clinical features, histopathological findings, treatment outcomes in 15 patients, who were diagnosed with HVLPD in the Department of Dermatology, Children's Hospital of Chongqing Medical University from 2014 to 2018, were retrospectively analyzed. Results Among the 15 patients, 7 were males and 8 were females. The age of onset ranged from 2 to 13 years, and the average age was 6.5 years. Main clinical manifestations included facial edema, and papules, blisters, ulcers and crusts repeatedly occurring on both the sun-exposed parts such as the face and limbs and non-sun-exposed parts such as the trunk. Of the 15 patients, 13 had intermittent fever, 13 had hepatosplenomegaly, and 15 had lymphadenectasis. Moreover, kidney failure occurred in 1 patient, hemophagocytic syndrome occurred in 2, and lymphoma occurred in 1. Laboratory examination showed that 15 patients were all positive for Epstein-Barr virus (EBV)-immunoglobulin G (IgG) antibody, and all negative for EBV-IgM antibody. Additionally, high replication of EBV DNA was detected. Histopathological study of skin lesions showed mild to dense perivascular and periadnexal infiltration of lymphocytes in the dermis and subcutaneous tissue. Immunohistochemistry study showed positive staining for CD4 and CD8 in 13 of 15 patients, for CD56 in 7 of 15 patients, and for CD3 in 12 of 13 patients. Moreover, 9 of 11 cases were detected positive for T-cell intracellular antigen-1, 8 cases were all detected positive for granzyme B, and the Ki-67 proliferation index ranged from 3％to 50％in 11 of 12 cases. The treatment protocols were similar among the 15 patients. After the treatment, 2 patients showed stable disease condition, and 10 experienced relapse. During the follow-up, 2 patients developed hemophagocytic syndrome, 1 developed lymphoma, and the 3 patients finally died of the worsening condition after chemotherapy. Conclusions HVLPD is closely associated with chronic active EBV infection. Its clinical symptoms can be relieved after immunomodulatory therapies such as glucocorticoids, gamma globulin and interferon, and the clinical manifestations, treatment outcomes and prognosis vary greatly among different patients.

RESUMEN

Objective@#To analyze clinical features and prognosis of 15 children with hydroa vacciniforme-like lymphoproliferative disorder (HVLPD) .@*Methods@#Clinical features, histopathological findings, treatment outcomes in 15 patients, who were diagnosed with HVLPD in the Department of Dermatology, Children′s Hospital of Chongqing Medical University from 2014 to 2018, were retrospectively analyzed.@*Results@#Among the 15 patients, 7 were males and 8 were females. The age of onset ranged from 2 to 13 years, and the average age was 6.5 years. Main clinical manifestations included facial edema, and papules, blisters, ulcers and crusts repeatedly occurring on both the sun-exposed parts such as the face and limbs and non-sun-exposed parts such as the trunk. Of the 15 patients, 13 had intermittent fever, 13 had hepatosplenomegaly, and 15 had lymphadenectasis. Moreover, kidney failure occurred in 1 patient, hemophagocytic syndrome occurred in 2, and lymphoma occurred in 1. Laboratory examination showed that 15 patients were all positive for Epstein-Barr virus (EBV) -immunoglobulin G (IgG) antibody, and all negative for EBV-IgM antibody. Additionally, high replication of EBV DNA was detected. Histopathological study of skin lesions showed mild to dense perivascular and periadnexal infiltration of lymphocytes in the dermis and subcutaneous tissue. Immunohistochemistry study showed positive staining for CD4 and CD8 in 13 of 15 patients, for CD56 in 7 of 15 patients, and for CD3 in 12 of 13 patients. Moreover, 9 of 11 cases were detected positive for T-cell intracellular antigen-1, 8 cases were all detected positive for granzyme B, and the Ki-67 proliferation index ranged from 3% to 50% in 11 of 12 cases. The treatment protocols were similar among the 15 patients. After the treatment, 2 patients showed stable disease condition, and 10 experienced relapse. During the follow-up, 2 patients developed hemophagocytic syndrome, 1 developed lymphoma, and the 3 patients finally died of the worsening condition after chemotherapy.@*Conclusions@#HVLPD is closely associated with chronic active EBV infection. Its clinical symptoms can be relieved after immunomodulatory therapies such as glucocorticoids, gamma globulin and interferon, and the clinical manifestations, treatment outcomes and prognosis vary greatly among different patients.

RESUMEN

Hydroa vacciniforme-like lymphoproliferative disorder (HVLPD) is defined as a distinctive clinicopathological type of cutaneous lymphoma and a subset of patients with this disease exhibit the natural killer (NK)-cell phenotype. The HVLPD-NK cell phenotype may be difficult to distinguish from cutaneous natural killer T-cell lymphoma (CNKTL), as these two diseases share similar immunophenotypic markers. Therefore, the aim of the present study was to analyze the clinicopathological features of this rare disease and compare these features with those of CNKTL. The clinical, histopathological and molecular features of 5 patients with the HVLPD-NK cell phenotype and 11 patients with CNKTL were evaluated. As well as certain subtle histopathological differences, there marked differences the age, distribution of lesions and clinical course differed between patients with these two diseases. These results suggest that the HVLPD-NK cell phenotype should be classified as a separate disorder and treated accordingly.