ABSTRACT
En el presente estudio describimos y caracterizamos la distribución geográfica de los casos positivos confirmados a HTLV-1 y 2 de pacientes peruanos con diagnóstico presuntivo entre 2019 y 2021. De un total de 555 muestras positivas confirmadas, 546 (98,4%) fueron HTLV-1 y 9 (1,6%) HTLV-2. Además, 22 de 24 departamentos del Perú presentaron casos de HTLV-1, siendo los principales motivos de solicitud de confirmación diagnóstica: aspirante a donar sangre con prueba de tamizaje reactivo, sospecha de leucemia/linfoma y paraparesia espástica tropical. Los resultados reflejan que la identificación de los puntos críticos constituye una brecha persistente respecto al diagnóstico, siendo cruciales para reducir el número de nuevos casos en Perú.
In the present study we describe and characterize the geographic distribution of HTLV-1 and 2 positive cases from Peruvian patients with presumptive diagnosis 2019 - 2021. Of a total of 555 confirmed positive samples, 546 (98.4%) were HTLV-1 and 9 (1.6%) HTLV-2. In addition, 22 of 24 departments of Peru presented cases of HTLV-1. The main reasons for requesting a confirmatory diagnosis being: aspiring to donate blood with a reactive screening test, suspicion of leukemia/ lymphoma and tropical spastic paraparesis. The results reflect that the identification of critical points constitutes a persistent gap regarding the diagnosis, being crucial to reduce the number of new cases in Peru.
ABSTRACT
O vírus linfotrópico T humano tipo 1 (HTLV-1) foi o primeiro retrovírus humano descoberto, descrito pela primeira vez há 41 anos. Esse retrovírus está associado ao desenvolvimento de duas doenças graves: a leucemia/linfoma de células T do adulto (ATLL) e a mielopatia associada ao HTLV-1/paraparesia espástica tropical (HAM/TSP). Este trabalho teve como objetivo analisar as atualizações sobre o HTLV-1, destacando os aspectos clínicos, os avanços e as limitações no tratamento e na prevenção da infecção pelo HTLV-1. Para isso, foi realizada uma revisão integrativa, por meio de coleta de dados nas plataformas PubMed, LILACS e SciELO, entre março e abril de 2021. Foram incluídos 61 artigos de diferentes países. O Brasil foi o país com maior número de publicações na área: 12. Os resultados obtidos mostram que existem avanços importantes no que diz respeito ao tratamento e à prevenção da infecção pelo HTLV-1. No entanto, a falta de estudos específicos sobre o vírus, que abordem os aspectos clínicos da infecção, foi um fator limitante para este estudo, o que reforça a necessidade de investimento em novas pesquisas sobre o tema.
The Human T-lymphotropic Virus 1 (HTLV-1) was the first human retrovirus discovered, described for the first time 41 years ago. This retrovirus is associated with the development of two serious diseases: adult T-cell leukemia/lymphoma (ATLL) and tropical spastic paraparesis/HTLV-1-associated myelopathy (HAM/TSP). This study aimed to analyze the updates about HTLV-1, highlighting the clinical aspects, advances, and limitations in the treatment and prevention of HTVL-1 infection. To this end, an integrative review was carried out, with data collection on PubMed, LILACS, and SciELO platforms, between March and April 2021. A total of 61 articles from different countries were included. Brazil was the country with the largest number of publications in the area: 12. The results showed effective advances regarding treating and preventing HTLV-1 infection. However, the lack of specific studies about the virus, which address the clinical aspects of the infection, was a limiting factor for this study, which reinforces the need for investment in new research about this topic.
El virus linfotrópico T tipo 1 humano (HTLV-1) fue el primer retrovirus humano descubierto y se describió por primera vez hace 41 años. Este retrovirus está asociado con el desarrollo de dos enfermedades graves: leucemia/linfoma de células T del adulto (ATLL) e mielopatía asociada a HTLV-1/paraparesia espástica tropical (HAM/TSP). Este estudio tuvo como objetivo analizar las actualizaciones sobre HTLV-1, destacando los aspectos clínicos, los avances y limitaciones en el tratamiento y prevención de la infección por HTLV-1. Para ello, se realizó una revisión integradora, a través de la recolección de datos en las plataformas PubMed, LILACS y SciELO entre marzo y abril de 2021. Se incluyeron 61 artículos de diferentes países. Brasil fue el país con mayor número de publicaciones en el área: 12. Los resultados obtenidos muestran que existen avances efectivos en cuanto al tratamiento y prevención de la infección por HTLV-1. Sin embargo, la falta de estudios específicos sobre el virus que aborden los aspectos clínicos de la infección fue un factor limitante para el presente estudio, lo que refuerza la necesidad de invertir en nuevas investigaciones sobre este virus.
Subject(s)
Human T-lymphotropic virus 1 , Deltaretrovirus Infections , Endogenous RetrovirusesABSTRACT
Adult T- lymphocyte leukemia/ lymphoma (ATLL), described by Uchiyama et al. in 1977, is a distinct neoplasia of peripheral T-lymphocytes caused by human T-cell lymphotropic virus type 1 (HTLV-1). The authors describe the case of a 75-year-old female patient who presented with fever, chills, and altered mental status. The peripheral blood morphology showed large atypical lymphocytes with multilobed nuclei and flow cytometry consistent with ATLL. The authors discuss the pathophysiology, differential diagnosis, and subtypes of ATLL in addition to the diagnostic approach using flow cytometry when bone marrow biopsy is not available and modalities of treatment.
Subject(s)
Humans , Female , Aged , Human T-lymphotropic virus 1 , Leukemia-Lymphoma, Adult T-Cell , Diagnosis, Differential , Flow CytometryABSTRACT
La leucemia/linfoma de células T adulto (LLCTA) es una neoplasia linfoide causada por el virus linfotrópico humano de células T tipo 1 (HTLV-1), retrovirus endémico en nuestro país. Tiene una presentación heterogénea, con variedades de pobre pronóstico e infecciones oportunistas que lo ensombrecen aún más. Se presentan dos casos de pacientes con variedades agresivas de la enfermedad (aguda y linfomatosa) complicadas con criptococosis sistémica. Reforzamos la necesidad de un enfoque diagnóstico completo y un monitoreo continuo de agentes oportunistas en el paciente con LLCTA adaptado a nuestro contexto epidemiológico
Adult T-cell leukemia/lymphoma (ATLL) is a lymphoid neoplasm caused by the human T-cell lymphotropic virus type 1 (HTLV-1), an endemic retrovirus in our country. It has a heterogeneous presentation, with sub types of poor prognosis, and opportunistic infections that worsen the prognosis. We present two cases with aggressive varieties of the disease (acute and lymphomatous) complicated by systemic cryptococcosis. It is necessary to conduct a complete diagnostic approach and continuous monitoring of opportunistic agents in patients with ATL adapted to our epidemiological context.
ABSTRACT
Objective@#To investigate the clinical presentation, clinicopathologic features, diagnosis and differential diagnosis of adult T cell leukemia/lymphoma (ATLL).@*Methods@#Four cases of ATLL from Fujian Cancer Hospital between October 2017 and May 2018 were analyzed using hematoxylin-eosin and immunohistochemical stains and polymerase chain reaction (PCR) for HTLV-1 provirus genes. The relevant literature was reviewed.@*Results@#There were two males and two females, age range 38-80 years. All patients were from coastal cities of Fujian province. Clinical presentations including lymphadenopathy, hepatomegaly and splenomegaly were detected in most patients; skin lesion, hypercalcemia and lymphocytosis were also commonly detected.Histologically, there was diffuse effacement of the normal architecture by tumor cells infiltration. The inflammatory background is usually sparse, with scanty eosinophils. The atypical lymphoid cells were typically medium to large sized with pronounced nuclear pleomorphism, irregular nuclei, chromatin clumping and prominent nucleoli. Blast-like cells with transformed nuclei were present in variable proportions. Giant cells with convoluted or cerebriform nuclear contours may be present. Rare cases may be composed predominantly of anaplastic tumor cells. Characteristic "flower cells" with large multi-lobated nuclei can be seen. The tumor cells were strongly positive for CD2, CD3, CD5, CD4 and CD25, but negative for CD7, CD8 and cytotoxic molecules (including TIA-1, Granzyme B and perforin). In three cases, the large transformed cells were positive for CD30. In one case, the anaplastic large cells were diffusely and strongly positive for CD30. All cases were negative for EBER, but positive for HTLV-1 provirus.@*Conclusions@#ATLL is a rare type of T cell lymphoma with unique clinical and pathological features, and should be distinguished from peripheral T cell lymphoma, NOS, ALK negative anaplastic large cell lymphoma and mycosis fungoides. Hypercalcemia, systemic disease, characteristic "flower cells" and specific immunophenotypic profile of CD3+, CD4+, CD25+, and CD7- are highly suggestive. However, ATLL can only be confirmed if the presence of HTLV-1 provirus.
ABSTRACT
La falla hepática aguda es el desarrollo de injuria hepática severa con deterioro de la función de síntesis y encefalopatía. Dentro de la variedad de causas, las infiltraciones neoplásicas representan menos del 0,5%. Presentamos el caso de un paciente varón HTLV1 positivo que debuta con una clínica de hepatitis aguda, siendo posteriormente diagnosticado con diseminación de linfoma/leucemia de células T del adulto. Desafortunadamente el paciente presentó deterioro rápido y progresivo de la función hepática, falleciendo a los pocos días de la hospitalización
Acute liver failure is the development of severe hepatic injury with deterioration of liver synthesis function and encephalopathy. Among all the variety of causes, neoplastic infiltration represents less than 0.5%. We present the case of a male patient with a past medical history of HTLV-1 infection, who reports symptoms of acute hepatitis, being diagnosed with Adult T-cell leukemia/ lymphoma. Unfortunately, the patient had a rapid deterioration and passed away a few days after admission
Subject(s)
Humans , Male , Middle Aged , HTLV-I Infections/complications , Leukemia-Lymphoma, Adult T-Cell/diagnosis , Liver Failure, Acute/etiology , Leukemia-Lymphoma, Adult T-Cell/complications , Leukemia-Lymphoma, Adult T-Cell/virology , Fatal OutcomeABSTRACT
Abstract: Infective dermatitis associated with HTLV-1 (IDH) is the main cutaneous marker of HTLV-1 infection. This disease occurs primarily in children and should be differentiated from other eczemas, especially from atopic dermatitis. The largest series of IDH are from Jamaica and Brazil. There are an estimated 15 to 20 million infected people in the world, and Brazil is one of the endemic regions. Studies suggest that IDH in children may be a marker for the development of T-cell leukemia/lymphoma (ATL) or myelopathy associated with HTLV-1/tropical spastic paraparesis (HAM / TSP) in adulthood.
Subject(s)
Humans , Female , Young Adult , HTLV-I Infections/diagnosis , Skin Diseases, Viral/diagnosis , Dermatitis/diagnosis , HTLV-I Infections/complications , Leukemia-Lymphoma, Adult T-Cell/diagnosis , Skin Diseases, Viral/complications , Dermatitis/virology , Dermatitis, Atopic/diagnosis , Dermatitis, Atopic/virology , Diagnosis, Differential , Eczema/diagnosis , Eczema/virologyABSTRACT
La infección por el virus linfotrópico de células T humanas tipo 1 (HTLV-1), es causante de la leucemia/linfoma de células T del Adulto (ATLL), siendo la afectación duodenal poco usual. La coinfección de HTLV-1 con Strongyloides stercolaris es común en los pacientes con HTLV- 1 debido a la inadecuada respuesta TH2 que presentan estos sujetos. Describimos a una paciente mujer de 48 años de edad, natural y procedente de la selva del Perú con historia familiar de infección por HTLV- 1 quien acude con diarrea crónica y baja de peso. Se le diagnosticó infección por HTLV-1 así como ATLL duodenal y estrongiloidiasis. Se inició tratamiento con ivermectina y quimioterapia, siendo estabilizada y dada de alta. Reportamos el presente caso debido a la poca frecuencia de coexistencia en duodeno de ATLL y estrongiloidiasis.
Infection by the Human T- Lymphotropic virus I (HTLV-1) causes Adult T cell Leukemia-lymphoma (ATLL), being the duodenal involvement rare. Commonly, patients co-infected with HTLV-1 and Strongyloides stercoralis are seen due to the lack of TH2 response found on these patients. We describe a 48-year-old woman, from the jungle of Peru, with a family history of HTLV-1 infection, who presented with a History of chronic diarrhea and weight loss. HTLV-1 infection with ATLL and strongyloidiasis were diagnosed. Ivermectin treatment and chemotherapy were initiated, being stabilized, and discharged. We report this case because of the unusual coexistence in the duodenum of ATLL and strongyloidiasis.
Subject(s)
Animals , Female , Humans , Middle Aged , Strongyloidiasis/diagnosis , HTLV-I Infections/diagnosis , Leukemia-Lymphoma, Adult T-Cell/diagnosis , Strongyloides stercoralis/isolation & purification , Duodenal Neoplasms/diagnosis , Coinfection/diagnosis , Duodenal Neoplasms/parasitology , Duodenal Neoplasms/virologyABSTRACT
Objective To develop allele specific oligonucleotide(ASO) -PCR assay based on TCR βgene rearrangements and provide a screening method for minimal residual disease (MRD) in adult patients with T-lineage acute lymphoblastic leukemia (T-ALL).Methods DNA samples from newly diagnosed 20 adult T-ALL patients were obtained.The TCR β gene rearrangements were detected by multiplex PCR,which included 38 paired of primers in 3 reaction tubes.Gel electrophoresis and two-color Gene Scanning was also applied for clonality analysis of TCR β followed by sequencing and subsequent blasting for monoclonal PCR products in four patients.ASO primers were designed based on the sequence of junction regions.MRD were detected in the bone marrow by RQ-PCR with ASO upstream primers, consensus Jβprobes and downstream primers.Results The detection rate of the clonal TCR β rearrangements was 85.0% (17/20).At least one complete Vβ-Jβ rearrangement could be detected at the time of diagnosis in 16 out of 17 patients(94.1%, 16/17).Incomplete Dβ-Jβ rearrangement could be detected in 7 patients (41.2% ,7/17).The positivitity rate of Vβ-Jβ to Dβ-Jβ was 2∶1 (94.1% versus 41.2% ).Two-color Gene Scanning analysis showed the Jβ2 family was used more frequently than the Jβ1 family (73% versus 27% ).The slopes of the standard curves ranged from - 3.60 to - 3.27.The correlation coefficients of all four standard curves were more than 0.99.The detection sensitivity of ASO-PCR was 4 × 10 -5 μg/μl.The fluorescence background were detected at a low level.Quantitative MRD values of TCR β rearrangement in sequential BM specimens of 4 adult T-ALL patients were monitored during the treatment, including complete remission after induction and after consolidation therapy. RQ-PCR showed the MRD values of TCR β rearrangement were gradually decreased in response to the treatment.Conclusions The quantification of TCR β rearrangement by ASO-PCR approach is sensitive, specific and reliable for the accurate evaluation of malignant clones.It is suitable for the monitoring of minimal residual disease of adult T-ALL patients.
ABSTRACT
We experienced a case of adult T cell leukemia/lymphoma (ATLL) in a 48-year-old Korean female, who has never been abroad since birth and no history of blood transfusion. The patient had hypercalcemia and multiple lymphadenopathy. Histopathologic study of left cervical lymph node (LN) and bone marrow (BM) revealed that infiltrates of malignant lymphoid cells were composed of small, medium and large cells with pleomorphic nuclei. Smears of peripheral blood (PB) showed lymphopenia (16%) with the appearance of a few atypical lymphoid cells (less than 2%), but not the typical clover leaf cells seen in ATLL. Immunophenotypic study of LN and BM revealed T cell phenotype. PB showed increased CD4+ T cell (T(H), CD3/CD4+, 57%) and decreased CD8+ T cell counts (T(S), CD3/CD8+, 6.7%). The sera of the patient and her family were reactive for HTLV-I antibody. The specific sequences of pol, env, and tax of HTLV-I DNA were detected in the lymphoma cells and peripheral blood mononuclear cells (PBMC) using polymerase chain reaction. Ultrastructural examination of PBMC confirmed numerous type c virus particles in extracellular space. This case was an acute type of ATLL without overt leukemic features in PB. Despite chemotherapy and intensive conservative treatment, she died 3 months after admission.