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1.
BMC Infect Dis ; 21(1): 17, 2021 Jan 06.
Artigo em Inglês | MEDLINE | ID: mdl-33407199

RESUMO

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.


Assuntos
Medula Óssea/patologia , Infecções por Vírus Epstein-Barr/complicações , Infecções por Vírus Epstein-Barr/diagnóstico , Herpesvirus Humano 4/genética , Hidroa Vaciniforme/complicações , Hidroa Vaciniforme/diagnóstico , Fígado/patologia , Transtornos Linfoproliferativos/complicações , Transtornos Linfoproliferativos/diagnóstico , Adulto , Antivirais/uso terapêutico , Pequim , Medula Óssea/virologia , Infecções por Vírus Epstein-Barr/tratamento farmacológico , Infecções por Vírus Epstein-Barr/virologia , Exantema/complicações , Exantema/tratamento farmacológico , Feminino , Hepatomegalia/tratamento farmacológico , Hepatomegalia/virologia , Humanos , Hidroa Vaciniforme/tratamento farmacológico , Hidroa Vaciniforme/patologia , Fígado/virologia , Linfoma de Células T/complicações , Linfoma de Células T/tratamento farmacológico , Linfoma de Células T/virologia , Transtornos Linfoproliferativos/tratamento farmacológico , Transtornos Linfoproliferativos/patologia , Pele/patologia , Resultado do Tratamento
4.
Am J Surg Pathol ; 44(10): 1340-1352, 2020 10.
Artigo em Inglês | MEDLINE | ID: mdl-32554995

RESUMO

Monomorphic posttransplant lymphoproliferative disorders have been defined as lymphoid or plasmacytic proliferations that fulfill criteria for one of the B-cell or T/NK-cell neoplasms recognized in immunocompetent hosts in the current WHO Classification. Low-grade B-cell neoplasms have historically been excluded from this category, although rare reports of marginal zone lymphoma (MZL) have been described. We report 9 cases of posttransplant Epstein-Barr virus-negative MZL, all arising in solid organ transplant recipients (4 renal, 3 liver, 1 cardiac, and 1 liver, pancreas, and small bowel). Seven were extranodal MZL of mucosa-associated lymphoid tissue type, all of which had gastrointestinal involvement (4 colon, 1 duodenum, 1 stomach, and 1 oropharynx/base of tongue). Notably, the preferential involvement of intestine distinguishes posttransplant extranodal MZL from sporadic cases. Immunoglobulin light-chain restriction was seen in all cases, with polymerase chain reaction showing a monoclonal pattern in 7 of 8 cases with successful amplification of polymerase chain reaction products. A clonally unrelated recurrence was seen in one case. Next-generation sequencing identified recurrent mutations previously reported in MZL in 3/5 cases. MZL was diagnosed at least 1 year after solid organ transplant (median time to presentation, 84 mo; range, 13 to 108 mo). The median age was 44 (range, 9 to 73 y); the male: female ratio was 5:4. The mean follow-up was 33.4 months, with an indolent clinical course observed. A subset responded to reduction in immunosuppression and anti-CD20 therapy alone. These data support the designation of Epstein-Barr virus-negative MZL as an uncommon form of monomorphic posttransplant lymphoproliferative disorders.


Assuntos
Hospedeiro Imunocomprometido , Linfoma de Zona Marginal Tipo Células B/imunologia , Transplante de Órgãos/efeitos adversos , Adolescente , Adulto , Idoso , Criança , Infecções por Vírus Epstein-Barr , Feminino , Humanos , Linfoma de Zona Marginal Tipo Células B/patologia , Transtornos Linfoproliferativos/imunologia , Transtornos Linfoproliferativos/patologia , Masculino , Pessoa de Meia-Idade
5.
Int J Hematol ; 112(5): 734-740, 2020 Nov.
Artigo em Inglês | MEDLINE | ID: mdl-32529584

RESUMO

Human herpesvirus type 8 (HHV8) is a gamma herpesvirus known for its role in lymphoid neoplasms, especially in immunosuppressed patients. We describe the case of a 64-year-old male, without known immunodeficiency, with 1-year-long clinical history of mediastinal and abdominal lymphadenopathies and recurrent pulmonary infections. Histopathological evaluation of a mediastinal lymph node revealed the presence of scattered atypical large cells with Hodgkin and Reed-Sternberg morphology in a background of lymphocytes and extensive areas of fibrosis. The large cells were positive for HHV8 and Epstein-Barr virus (EBV), with a clonal pattern of IGH gene rearrangement. A descriptive diagnosis of "HHV8-positive, EBV-positive Hodgkin lymphoma-like large B-cell lymphoma" was rendered. Interestingly, the retrospective evaluation of a previous biopsy, diagnosed as reactive lymphadenitis, revealed the presence of HHV8- and EBV-positive cells, with a polyclonal pattern and a small peak corresponding to that of the most recent biopsy. This case presents diagnostic challenges due to the presence of particular features not clearly related to current HHV8-associated entities, and also suggests the possibility for disease progression in the spectrum of HHV8- and EBV-associated lymphoproliferative disorders.


Assuntos
Herpesvirus Humano 4 , Herpesvirus Humano 8 , Doença de Hodgkin/patologia , Doença de Hodgkin/virologia , Linfoma Difuso de Grandes Células B/patologia , Linfoma Difuso de Grandes Células B/virologia , Transtornos Linfoproliferativos/patologia , Transtornos Linfoproliferativos/virologia , Progressão da Doença , Humanos , Hospedeiro Imunocomprometido , Linfonodos/patologia , Linfonodos/virologia , Masculino , Pessoa de Meia-Idade , Células de Reed-Sternberg/patologia , Células de Reed-Sternberg/virologia
6.
Crit Rev Oncol Hematol ; 151: 102981, 2020 Jul.
Artigo em Inglês | MEDLINE | ID: mdl-32485429

RESUMO

The topic of fertility preservation in patients with a lymphoproliferative disease offers new aspects of debate, due to the introduction of novel chemotherapeutic regimens and small molecules in the clinical landscape. Cancer related infertility is mostly dependent on gonadotoxic treatments and fertile female patients are today addressed to the oocyte cryopreservation or to ovarian cortex fragment cryopreservation. These methods present advantages and disadvantages, which will be discussed in the present review, together with the options for male patients. The recent discovery of functional ovarian stem cells (OCSs) in woman ovarian cortex, opens new avenues offering a innovative procedure for fertility preservation through as model of regenerative medicine. Here, we review the gonadotoxic potential of "classical" chemotherapeutic treatments as well as of "novel" targeted therapies actually employed for lymphoproliferative neoplasms in young patients and revisit both the today available and future chances to preserve and restore fertility after the cancer healing.


Assuntos
Antineoplásicos/efeitos adversos , Preservação da Fertilidade , Fertilidade/efeitos dos fármacos , Transtornos Linfoproliferativos/dietoterapia , Neoplasias/tratamento farmacológico , Ovário/efeitos dos fármacos , Insuficiência Ovariana Primária/induzido quimicamente , Criopreservação , Feminino , Humanos , Transtornos Linfoproliferativos/patologia , Masculino , Oócitos
7.
Exp Hematol ; 86: 67-77.e2, 2020 06.
Artigo em Inglês | MEDLINE | ID: mdl-32422231

RESUMO

There exists an urgent need for the development of new drugs for the treatment of lymphoid neoplasms. The aim of this study was to evaluate the cytotoxic effect of the marine plastoquinone 9'-hydroxysargaquinone (9'-HSQ), focusing on investigation of the mechanism by which it causes death in lymphoid neoplastic cells. This particular plastoquinone reduced the cell viability of different hematological tumor cell lines in a time-dependent and concentration-dependent manner. Intrinsic apoptosis occurred with time-dependent reduction of mitochondrial membrane potential (42.3 ± 1.1% of Daudi cells and 18.6 ± 5.6% of Jurkat cells maintained mitochondrial membrane integrity) and apoptosis-inducing factor release (Daudi: 133.3 ± 8.1%, Jurkat: 125.7 ± 6.9%). Extrinsic apoptosis also occurred, as reflected by increased FasR expression (Daudi: 139.5 ± 7.1%, Jurkat: 126.0 ± 1.0%). Decreases were observed in the expression of Ki-67 proliferation marker (Daudi: 67.5 ± 2.5%, Jurkat: 84.3 ± 3.8%), survivin (Daudi: 66.0 ± 9.9%, Jurkat: 63.1 ± 6.0%), and NF-κB (0.7 ± 0.04% in Jurkat cells). Finally, 9'-HSQ was cytotoxic to neoplastic cells from patients with different lymphoid neoplasms (IC50: 4.9 ± 0.6 to 34.2 ± 0.4 µmol/L). These results provide new information on the apoptotic mechanisms of 9'-HSQ and suggest that it might be a promising alternative for the treatment of lymphoid neoplasms.


Assuntos
Antineoplásicos/farmacologia , Apoptose/efeitos dos fármacos , Organismos Aquáticos/química , Neoplasias Hematológicas/tratamento farmacológico , Transtornos Linfoproliferativos/tratamento farmacológico , Feófitas/química , Plastoquinona/farmacologia , Antineoplásicos/química , Ensaios de Seleção de Medicamentos Antitumorais , Neoplasias Hematológicas/metabolismo , Neoplasias Hematológicas/patologia , Humanos , Células Jurkat , Células K562 , Transtornos Linfoproliferativos/metabolismo , Transtornos Linfoproliferativos/patologia , Plastoquinona/química
8.
Int J Pediatr Otorhinolaryngol ; 134: 110066, 2020 Jul.
Artigo em Inglês | MEDLINE | ID: mdl-32361254

RESUMO

Tonsillectomy is a common procedure in the pediatric population, with subsequent microscopic examination of the specimen for cancer and other rare diagnoses occurring routinely. A 17 year-old female with a history of autoimmune vasculitis underwent adenotonsillectomy for severe obstructive sleep apnea. Pathology demonstrated small, medium and large lymphocytes and plasma cells obscuring the lymphoid follicles and germinal centers, with few Epstein-Barr virus positive lymphocytes. Tingible body macrophages were seen in the vaguely nodular areas. This reactive histologic pattern represents an atypical lymphoproliferative disorder never before documented in tonsils. Histopathologic images will be shown.


Assuntos
Transtornos Linfoproliferativos/patologia , Tonsila Palatina/patologia , Apneia Obstrutiva do Sono/cirurgia , Tonsilectomia , Adenoidectomia , Adolescente , Feminino , Humanos , Hiperplasia , Linfócitos/patologia , Tonsila Palatina/imunologia
10.
Lancet Haematol ; 7(6): e479-e489, 2020 Jun.
Artigo em Inglês | MEDLINE | ID: mdl-32470439

RESUMO

Mature lymphoproliferative diseases are a heterogeneous group of neoplasms arising from different stages of B-cell and T-cell development. With improved understanding of the molecular processes in lymphoma and novel treatment options, arises a growing need for the molecular characterisation of tumours. Molecular imaging with single-photon-emission CT and PET using specific radionuclide tracers can provide whole-body information to investigate cancer biology, to evaluate phenotypic heterogeneity, to identify resistance to targeted therapy, and to assess the biodistribution of drugs in patients. In this Review, we evaluate the existing literature on molecular imaging in lymphoma, other than 18F-fluordeoxyglucose molecular imaging. The aim is to examine the contribution of molecular imaging to the understanding of the biology of lymphoma and to discuss potential implications for the diagnostics and therapy of this disease. Finally, we discuss possible applications for molecular imaging of patients with lymphoma in the clinical context.


Assuntos
Fluordesoxiglucose F18/metabolismo , Linfoma/diagnóstico por imagem , Imagem Molecular/métodos , Biomarcadores Tumorais/metabolismo , Ensaios Clínicos como Assunto , Humanos , Imunoterapia/métodos , Linfoma/terapia , Transtornos Linfoproliferativos/patologia , Estadiamento de Neoplasias/métodos , Tomografia por Emissão de Pósitrons/métodos , Medicina de Precisão/métodos , Radioimunoterapia/métodos , Radioisótopos/metabolismo , Distribuição Tecidual/efeitos dos fármacos
11.
Blood Cancer J ; 10(4): 42, 2020 04 22.
Artigo em Inglês | MEDLINE | ID: mdl-32321919

RESUMO

The molecular pathogenesis of chronic lymphoproliferative disorder of natural killer (NK) cells (CLPD-NK) is poorly understood. Following the screening of 57 CLPD-NK patients, only five presented STAT3 mutations. WES profiling of 13 cases negative for STAT3/STAT5B mutations uncovered an average of 18 clonal, population rare and deleterious somatic variants per patient. The mutational landscape of CLPD-NK showed that most patients carry a heavy mutational burden, with major and subclonal deleterious mutations co-existing in the leukemic clone. Somatic mutations hit genes wired to cancer proliferation, survival, and migration pathways, in the first place Ras/MAPK, PI3K-AKT, in addition to JAK/STAT (PIK3R1 and PTK2). We confirmed variants with putative driver role of MAP10, MPZL1, RPS6KA1, SETD1B, TAOK2, TMEM127, and TNFRSF1A genes, and of genes linked to viral infections (DDX3X and RSF1) and DNA repair (PAXIP1). A truncating mutation of the epigenetic regulator TET2 and a variant likely abrogating PIK3R1-negative regulatory activity were validated. This study significantly furthered the view of the genes and pathways involved in CLPD-NK, indicated similarities with aggressive diseases of NK cells and detected mutated genes targetable by approved drugs, being a step forward to personalized precision medicine for CLPD-NK patients.


Assuntos
Biomarcadores Tumorais/genética , Evolução Clonal , Células Matadoras Naturais/patologia , Transtornos Linfoproliferativos/genética , Transtornos Linfoproliferativos/patologia , Mutação , Fator de Transcrição STAT3/genética , Adulto , Idoso , Feminino , Humanos , Células Matadoras Naturais/metabolismo , Masculino , Pessoa de Meia-Idade , Sequenciamento Completo do Exoma/métodos
12.
Am J Surg Pathol ; 44(8): 1061-1072, 2020 08.
Artigo em Inglês | MEDLINE | ID: mdl-32317607

RESUMO

Systemic Epstein-Barr virus-positive T-cell and natural killer (NK)-cell lymphoproliferative diseases of childhood are a group of lethal diseases mostly affecting children and young adults. The Ohshima Grading System and the 2017 World Health Organization (WHO) classification have been used for classifying this spectrum, but these systems have not been validated externally and compared. Therefore, we examined 36 cases of systemic Epstein-Barr virus-positive T-cell and NK-cell lymphoproliferative diseases of childhood with long-term follow-up, from Southwest China, to systematically summarize the clinicopathologic features and to validate and compare the Ohshima Grading System and the 2017 WHO classification in discrimination ability, predictive accuracy, concordance indices, and explained variation. Clinically, our cohort showed severe manifestations and poor prognoses. Morphologically, the hematopoietic and lymphoid specimens showed proliferation of small-sized to medium-sized bland-looking lymphocytes that might mask disease severity, whereas other extranodal lesions showed a disorganized to obliterated architecture infiltrated by medium-sized to large-sized, subtle to obvious atypical cells, which may mimic extranodal NK/T-cell lymphoma. Immunophenotypically, our cases mainly originate from CD8 αß T cells. Therefore, clinical and pathologic features should be equally considered to avoid missed diagnosis or misdiagnosis. In addition, the 2017 WHO classification shows a flexible grasp of pathologic features, thus classifying some cases (polymorphic and monoclonal cases with fulminant course) more reasonably; thereby, it showed statistically improved results compared with the Ohshima Grading System. However, underestimating the risk of some polyclonal cases and imprecisely discriminating monoclonal cases at diagnosis are common dilemmas in both systems. Therefore, the construction of a comprehensive grading algorithm for improved prognostic value and precise diagnosis requires additional studies.


Assuntos
Infecções por Vírus Epstein-Barr/classificação , Herpesvirus Humano 4/genética , Células Matadoras Naturais/imunologia , Transtornos Linfoproliferativos/classificação , Linfócitos T/imunologia , Adolescente , Adulto , Idade de Início , Proliferação de Células , Criança , Pré-Escolar , China , Bases de Dados Factuais , Infecções por Vírus Epstein-Barr/imunologia , Infecções por Vírus Epstein-Barr/patologia , Infecções por Vírus Epstein-Barr/virologia , Feminino , Genes Codificadores da Cadeia alfa de Receptores de Linfócitos T , Genes Codificadores da Cadeia beta de Receptores de Linfócitos T , Humanos , Lactente , Células Matadoras Naturais/patologia , Transtornos Linfoproliferativos/imunologia , Transtornos Linfoproliferativos/patologia , Transtornos Linfoproliferativos/virologia , Masculino , Pessoa de Meia-Idade , Prognóstico , RNA Viral/genética , Estudos Retrospectivos , Medição de Risco , Fatores de Risco , Linfócitos T/patologia , Fatores de Tempo , Adulto Jovem
13.
Anticancer Res ; 40(4): 2019-2023, 2020 Apr.
Artigo em Inglês | MEDLINE | ID: mdl-32234892

RESUMO

BACKGROUND/AIM: CD38 is a cell surface marker commonly present in plasma cells and activated T cells, while CD138 is a representative plasma cell marker. The aim of this study was to describe the expression of cell surface markers including CD38 and CD138, in the tumors of patients with IgG4-related ophthalmic disease (IgG4-ROD) and extranodal marginal zone B-cell lymphoma (EMZL) of the ocular adnexa. MATERIALS AND METHODS: Twenty-four consecutive patients of whom 12 had IgG4-ROD and 12 EMZL were enrolled in this study. Medical records were reviewed for flow cytometry (FCM) results on conventional T-cell markers, B-cell markers, CD38 and CD138. RESULTS: Positive rates of T-cell markers, CD38 and CD138 were significantly higher in IgG4-ROD than in EMZL (p<0.01 and p<0.05, respectively). CONCLUSION: Our FCM results on CD38 and CD138 showed that the lymphocyte populations were different between IgG4-ROD and EMZL, which may reflect the different pathophysiology of the two diseases.


Assuntos
ADP-Ribosil Ciclase 1/sangue , Doença Relacionada a Imunoglobulina G4/sangue , Linfoma de Zona Marginal Tipo Células B/sangue , Transtornos Linfoproliferativos/sangue , Sindecana-1/sangue , Idoso , Linfócitos B/patologia , Biomarcadores Tumorais/sangue , Linhagem da Célula/genética , Feminino , Citometria de Fluxo , Humanos , Doença Relacionada a Imunoglobulina G4/genética , Doença Relacionada a Imunoglobulina G4/patologia , Linfoma de Zona Marginal Tipo Células B/genética , Linfoma de Zona Marginal Tipo Células B/patologia , Transtornos Linfoproliferativos/genética , Transtornos Linfoproliferativos/patologia , Masculino , Pessoa de Meia-Idade , Linfócitos T/patologia
15.
Hum Genet ; 139(6-7): 885-901, 2020 Jun.
Artigo em Inglês | MEDLINE | ID: mdl-32152698

RESUMO

Epstein-Barr virus (EBV) is a ubiquitous human pathogen, infecting > 90% of the adult population. In the vast majority of healthy individuals, infection with EBV runs a relatively benign course. However, EBV is by no means a benign pathogen. Indeed, apart from being associated with at least seven different types of malignancies, EBV infection can cause severe and often fatal diseases-hemophagocytic lymphohistiocytosis, lymphoproliferative disease, B-cell lymphoma-in rare individuals with specific monogenic inborn errors of immunity. The discovery and detailed investigation of inborn errors of immunity characterized by heightened susceptibility to, or increased frequency of, EBV-induced disease have elegantly revealed cell types and signaling pathways that play critical and non-redundant roles in host-defense against EBV. These analyses have revealed not only mechanisms underlying EBV-induced disease in rare genetic conditions, but also identified molecules and pathways that could be targeted to treat severe EBV infection and pathological consequences in immunodeficient hosts, or even potentially enhance the efficacy of an EBV-specific vaccine.


Assuntos
Infecções por Vírus Epstein-Barr/complicações , Predisposição Genética para Doença , Herpesvirus Humano 4/imunologia , Interações Hospedeiro-Patógeno/genética , Síndromes de Imunodeficiência/complicações , Transtornos Linfoproliferativos/etiologia , Infecções por Vírus Epstein-Barr/imunologia , Infecções por Vírus Epstein-Barr/virologia , Herpesvirus Humano 4/genética , Humanos , Síndromes de Imunodeficiência/imunologia , Síndromes de Imunodeficiência/virologia , Transtornos Linfoproliferativos/patologia , Transdução de Sinais
16.
Indian J Pathol Microbiol ; 63(1): 78-82, 2020.
Artigo em Inglês | MEDLINE | ID: mdl-32031127

RESUMO

Introduction: Epstein-Barr Virus (EBV)-associated systemic T-cell lymphoproliferative disorder of childhood is a rare but severe manifestation of chronic EBV infection. Despite several case reports characterizing this rare hematological neoplasm, the literature describes extensive heterogeneity in the presentation of this disease. Case presentation: Here we present a complete autopsy of a 16-year-old girl who ultimately succumbed to EBV-associated systemic T-cell lymphoproliferative disorder of childhood. Her clinical presentation demonstrated a non-specific pharyngitis with positive mono spot test, evolving into fulminant multi-organ failure, disseminated intravascular coagulopathy, sepsis, and ultimately death. Conclusions: Post-mortem findings included extensive hemorrhage, and infiltration of the liver, spleen, lymph nodes and bone marrow with neoplastic T-cells. There was extensive hemophagocytic lymphohistiocytosis (HLH) within these organs, suggesting overlap between the EBV-associated systemic T-cell lymphoproliferative disorder of childhood and EBV-associated HLH. We hope these findings provide a more comprehensive overview of several possible manifestations of EBV-associated systemic T-cell lymphoproliferative disorder of childhood.


Assuntos
Autopsia , Infecções por Vírus Epstein-Barr/patologia , Linfo-Histiocitose Hemofagocítica/patologia , Transtornos Linfoproliferativos/patologia , Adolescente , Biópsia , Medula Óssea/patologia , Infecções por Vírus Epstein-Barr/complicações , Infecções por Vírus Epstein-Barr/imunologia , Evolução Fatal , Feminino , Humanos , Linfonodos/patologia , Linfo-Histiocitose Hemofagocítica/virologia , Transtornos Linfoproliferativos/virologia , Insuficiência de Múltiplos Órgãos , Sepse , Linfócitos T/patologia
17.
Nephrol Dial Transplant ; 35(2): 336-345, 2020 02 01.
Artigo em Inglês | MEDLINE | ID: mdl-32030416

RESUMO

BACKGROUND: Belatacept (bela) rescue therapy seems to be a valuable option for calcineurin inhibitor chronic toxicity in kidney transplantation. Nevertheless, the risk of infection associated with bela is not well reported. METHODS: We report the rate of opportunistic infections (OPI) after a switch to bela in a multicentric cohort of 280 kidney transplant patients. RESULTS: Forty-two OPI occurred in 34 patients (12.1%), on average 10.8 ± 11.3 months after the switch. With a cumulative exposure of 5128 months of bela treatment, we found an incidence of 0.008 OPI/month of exposure, and 9.8 OPI/100 person-years. The most common OPI was cytomegalovirus (CMV) disease in 18/42 OPI (42.9%) and pneumocystis pneumonia in 12/42 OPI (28.6%). Two patients presented a progressive multifocal leucoencephalopathy and two patients developed a cerebral Epstein-Barr virus-induced post-transplant lymphoproliferative disease. OPI led to death in 9/34 patients (26.5%) and graft failure in 4/34 patients (11.8%). In multivariate analysis, estimated glomerular filtration rate <25/mL/min/1.73 m2 on the day of the switch and the use of immunosuppressive agents before transplantation were associated with the occurrence of OPI. We found a higher rate of infection-related hospitalization (24.1 versus 12.3/100 person-years, P = 0.0007) and also a higher rate of OPI (13.2 versus 6.7/100 person-years, P = 0.005) in the early conversion group (within 6 months). CONCLUSIONS: The risk of OPI is significant post-conversion to bela and may require additional monitoring and prophylactic therapy, particularly regarding pneumocystis pneumonia and CMV disease. These data need to be confirmed in a larger case-control study.


Assuntos
Abatacepte/efeitos adversos , Rejeição de Enxerto/tratamento farmacológico , Sobrevivência de Enxerto/efeitos dos fármacos , Imunossupressores/efeitos adversos , Transplante de Rim/efeitos adversos , Transtornos Linfoproliferativos/epidemiologia , Infecções Oportunistas/epidemiologia , Feminino , França/epidemiologia , Taxa de Filtração Glomerular , Rejeição de Enxerto/etiologia , Rejeição de Enxerto/patologia , Humanos , Incidência , Transtornos Linfoproliferativos/etiologia , Transtornos Linfoproliferativos/patologia , Masculino , Pessoa de Meia-Idade , Infecções Oportunistas/etiologia , Infecções Oportunistas/patologia , Estudos Retrospectivos
20.
Semin Diagn Pathol ; 37(1): 32-46, 2020 Jan.
Artigo em Inglês | MEDLINE | ID: mdl-31889602

RESUMO

The spectrum of Epstein-Barr virus (EBV)-positive T and NK-cell lymphoproliferations is broad and ranges from reactive self-limited disorders to neoplastic processes with a fulminant clinical course. EBV plays an important role promoting lymphomagenesis, although the precise mechanisms remain elusive. EBV-positive lymphoproliferative disorders (LPD) are more common in East Asia (China, Japan, Korea and Taiwan), and Latin America suggesting a strong genetic predisposition. The revised 2016 World Health Organization (WHO) lymphoma classification recognizes the following malignant NK- and T-cell lymphomas; extranodal NK/T-cell lymphoma, nasal type (ENKTCL), aggressive NK-cell leukemia (ANKL), and the provisional entity within the group of peripheral T-cell lymphoma, not otherwise specified (PTCL, NOS) "primary EBV-positive nodal T or NK cell lymphoma". Disorders presenting mainly in children and young adults include chronic active EBV infection (CAEBV) - systemic and cutaneous forms - which are not considered malignant disorders but were included in the WHO classification for the first time because of the differential diagnosis with other T- or NK-cell lymphomas. CAEBV, cutaneous form, includes hydroa vacciniforme-like LPD (HV-LPD) and severe mosquito bite allergy (SMBA). Finally, systemic EBV-positive T-cell lymphoma of childhood was recognized as lymphoma because of its fulminant clinical course. Given the shared pathogenesis of these disorders, overlapping features are common demanding a close clinical, morphological and molecular correlation for an accurate diagnosis. This review summarizes the clinical, histopathological and molecular features of EBV-associated T and NK-cell LPD, highlighting the main features that might aid in the differential diagnosis.


Assuntos
Infecções por Vírus Epstein-Barr/complicações , Células Matadoras Naturais/patologia , Transtornos Linfoproliferativos/patologia , Transtornos Linfoproliferativos/virologia , Linfócitos T/patologia , Humanos , Células Matadoras Naturais/virologia , Linfócitos T/virologia
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