Adult T-cell leukemia/lymphoma (ATLL)

2005-05-01   Gianluigi Castoldi , Antonio Cuneo 

1.Hematology Section, Department of Biomedical Sciences, University of Ferrara, Corso Giovecca 203, Ferrara, Italy

Clinics and Pathology

Phenotype stem cell origin

This is a T-cell lymphoid neoplasia caused by HTLV1 infection. The phenotype is CD3+, CD5+, CD7- with positivity for the CD4 and CD25 molecules in the majority of the cases.

Etiology

Pathogenesis of the disease: ATLL is associated with HTLV-1 infection of the tumour clone in 100% of the cases. The interval between HTLV-1 infection and the onset of lymphoma is long (10-40 years) and only <5% of infected people actually develops the disease. HTLV-1 produces a trans-regulatory protein (Tax) inducing interleukin-2 (IL-2) and IL-2 receptor expression and consequent polyclonal CD4 cell growth. This T-cell population is at risk for the development of genetic and cytogenetic changes leading to lymphoma.

Epidemiology

The disease affects adult people. Clusters were observed in Japan and in the Caribbean; sporadic cases were reported in Western countries.

Clinics

The disease usually runs an aggressive course, with peripheral blood and bone marrow involvement, diffuse adenopathies, hepatomegaly, bone lesions and hypercalcemia. Smouldering or chronic forms were also observed.

Pathology

The lymph node architecture is effaced by a diffuse proliferation of small and large lymphoid cells having pleomorphic cytological features. In the peripheral blood the neoplastic cells often display a lobated nucleus (flower cells); diffuse bone marrow infiltration is found in virtually all cases.

Treatment

Multiagent chemotherapy usually attains only partial, short lasting responses. Highly active anti viral therapy with zidouvidine and interferon-alpha may be beneficial in some cases.

Prognosis

Patients with aggressive disease usually survive less than 1 year; less than 10% of the patients survive more than 5 years. Longer survival (> 2 years) can be observed in rare patients presenting a chronic or a smouldering form.

Cytogenetics

Note

The karyotype almost invariably shows a high degree of complexity and variability. Aneuploidy and more than 6 chromosome breaks were observed in the majority of cases. The most frequent gains include trisomy 3, trisomy 8, trisomy 9 and trisomy 21; monosomies involve chromosome 4, 8, 10 and 22. Breakpoints clusters are found at 1p and 1q, at 3q, 6q, 7q, 10p, 12q, 13q, 14q, 17p and 21p. Multiple breaks and aberrations of some of these chromosome regions may predict for an inferior outcome.

Cytogenetics molecular

Comparative genomic hybridization (CGH) studies revealed that the most frequent regions of DNA gains are located at 14q, 7q and 3p; whereas frequent losses involve sequences at 6q and 13q. Gain of 14q32 may be a recurrent specific abnormality in ATLL. Aggressive forms display more genomic aberrations than chronic forms. The number of chromosomal imbalances correlates with clinical outcome. Different hybridization patterns, suggesting clonal evolution, can be observed when analysing material from different sites or material taken at different time points in the same patient.
Upregulation of gene encoding for ribosomal proteins, proteosome subunits, translation factors was identified in acute vs chronic phases of the disease. Many of these genes are located in regions amplified by chromosome rearrangements. Downregulation of genes involved in immune response was also documented.

Highly cited references

Pubmed IDYearTitleCitations
357347742022Serum miR-204 and miR-451 Expression and Diagnostic Value in Patients with Pulmonary Artery Hypertension Triggered by Congenital Heart Disease.0
357203362022Clinical Variables, Deep Learning and Radiomics Features Help Predict the Prognosis of Adult Anti-N-methyl-D-aspartate Receptor Encephalitis Early: A Two-Center Study in Southwest China.0
357180712022Development of a Deep Learning Model for the Histological Diagnosis of Dysplasia in Barrett's Esophagus.0
357177052022Predicting checkpoint inhibitors pneumonitis in non-small cell lung cancer using a dynamic online hypertension nomogram.0
357109522022Cost-effectiveness of Alzheimer's disease CSF biomarkers and amyloid-PET in early-onset cognitive impairment diagnosis.0
357020212022The development of a scale to assess children's and adolescents' sleep environments.0
356994442022Using Machine Learning To Define the Impact of Beta-Lactam Early and Cumulative Target Attainment on Outcomes in Intensive Care Unit Patients with Hospital-Acquired and Ventilator-Associated Pneumonia.0
356911022022Psychometric properties of a Mandarin Chinese version of the Body Appreciation Scale-2 with residents from Chinese mainland.0
356895382022Prognostic value of dynamic cardiac biomarkers in patients with acquired refractory thrombocytopenic purpura: A retrospective study in Chinese population.0
356849612022Multiplex Detection of Antibody Landscapes to SARS-CoV-2/Influenza/Common Human Coronaviruses Following Vaccination or Infection with SARS-CoV-2 and Influenza.0
356768872022Migraine with aura associates with a higher artificial intelligence: ECG atrial fibrillation prediction model output compared to migraine without aura in both women and men.0
356712462022DNA polymorphisms and expression profile of immune and antioxidant genes as biomarkers for reproductive disorders tolerance/susceptibility in Baladi goat.0
356587662022Duloxetine overdose causes sympathomimetic and serotonin toxicity without major complications.0
356551182022In silico identification of the rare-coding pathogenic mutations and structural modeling of human NNAT gene associated with anorexia nervosa.0
356523422022Association of Cardiovascular Health Through Young Adulthood With Genome-Wide DNA Methylation Patterns in Midlife: The CARDIA Study.0
356483252022Identification of QTLs and a putative candidate gene involved in rhizome enlargement of Asian lotus (Nelumbo nucifera).0
356443122022Effects of HIF-1α, hepcidin and PTH on RankL in patients with chronic kidney disease in different stages.0
356404322022Plasma lipoprotein-associated phospholipase A2 is associated with acute ischemic stroke in patients with atrial fibrillation.0
356403562022'Thinking about Them is only making me feel worse'. The mediating role of metacognitive factors in the relationship between paranoia-like beliefs and psychopathology symptoms in a community sample.0
355767632022Metagenomic profiling and transfer dynamics of antibiotic resistance determinants in a full-scale granular sludge wastewater treatment plant.0
355746172022Plasminogen Activator Inhibitor-1 Levels in Non-alcoholic Fatty Liver Disease: A Systematic Review and Meta-Analysis.0
355449702022Two-step micelle-to-solvent stacking of arsenic species from foods in permanently coated tubing for capillary electrophoresis.0
355026232022Programmed death-ligand 1 expression and use of immune checkpoint inhibitors among patients with advanced non-small-cell lung cancer in a resource-limited country.0
354506172022Graphene oxide quantum dots attached on wood-derived nanocellulose to fabricate a highly sensitive humidity sensor.0
354403972022Visualization of polypeptides including fragmented α-amylase in rice koji grains using mass spectrometry imaging.0
354289772022Scattered cerebral infarction in the corona radiata predicts worse outcomes.0
354218152022Standardized ileal digestibility of amino acids in broiler chickens fed single or mixture of feed ingredients-based diets with or without Eimeria challenge.0
353986432022Enhanced removal of neonicotinoid pesticides present in the Decision 2018/840/EU by new sewage sludge-based carbon materials.0
353893002022Respiratory failure and bioelectrical phase angle are independent predictors for long-term survival in acute heart failure.0
353009522022Does Surgical Trainee Participation Affect Infection Outcomes in Primary Total Knee Arthroplasty?0
352988932022Once-only colonoscopy or two rounds of faecal immunochemical testing 2 years apart for colorectal cancer screening (SCREESCO): preliminary report of a randomised controlled trial.1
352562832022COVID-19 outbreak impact on health professionals: A survey on the Italian radiographer experience.0
352399832022Assessment of masticatory function in the differential diagnosis of Stage IV periodontitis: A pilot diagnostic accuracy study.0
351775652022Performance Evaluation of the Aptima Assays in Comparison with the cobas 6800 Assays for the Detection of HIV-1, HBV, and HCV in Clinical Samples.0
348504022022Analysis of two laboratory tests for determination of EBV-IM in children.0
348396752022Trends in Mental Health Concerns Reported to Two Pediatric Mental Health Care Access Programs During the COVID-19 Pandemic.0
347919522022Pregnancy in women with MS: Impact on long-term disability accrual in a nationwide Danish Cohort.0
347276352022Identification of polymorphic loci in the deiodinase 2 gene and their associations with head dimensions in geese.0
347096722022The Value of Chinese Thyroid Imaging Report and Data System Combined With Contrast-Enhanced Ultrasound Scoring in Differential Diagnosis of Benign and Malignant Thyroid Nodules.0
345618722022Simultaneous use of high-volume Foley catheter (60 ml) and misoprostol for labor induction in nulliparous women: A randomized controlled trial.0
344534662022Effect of Scanner Type and Scan Body Location on the Accuracy of Mandibular Complete-Arch Digital Implant Scans: An In Vitro Study.0
339733842022General Safety and Tolerability of Subcutaneous Tanezumab for Osteoarthritis: A Pooled Analysis of Three Randomized, Placebo-Controlled Trials.0
356932312022Polymerization kinetics of bicyclic olefins and mechanism with symmetrical ansa-metallocene catalysts associated with active center count: relationship between their activities and structure and activation path.0
356923232022Analysis of the Impacts on the Psychological Changes of Chinese Returning College Students After the Outbreak of the 2019 Coronavirus Disease.0
356838032022Poly-ε-Caprolactone-Hydroxyapatite-Alumina (PCL-HA-α-Al2O3) Electrospun Nanofibers in Wistar Rats.0
356716182022The beneficial cutoffs of vitamin D for metabolic syndrome varies by sex among the elderly Chinese population: A cross-sectional study.0
356637352022Characterization, kinetics and thermodynamics of epoxidation-esterification of Irvingia gabonensis kernel oil methyl ester.0
356439442022Association between cardiovascular health metrics and risk of incident type 2 diabetes mellitus: the Rural Chinese Cohort Study.0
356434362022Fasting serum fructose is associated with risk of gestational diabetes mellitus.0
356364442022Upfront FOLFOXIRI plus bevacizumab with or without atezolizumab in the treatment of patients with metastatic colorectal cancer (AtezoTRIBE): a multicentre, open-label, randomised, controlled, phase 2 trial.0
356297392022Mechanical Behavior and Structural Characterization of a Cu-Al-Ni-Based Shape-Memory Alloy Subjected to Isothermal Uniaxial Megaplastic Compression.0
356202512022Evaluation of morning bradykinesia in Parkinson's disease in a United States cohort using continuous objective monitoring.0
356120102022AP-HP Health Data Space (AHDS) to the Test of the Covid-19 Pandemic.0
356090582022Methodological implications of sample size and extinction gradient on the robustness of fear conditioning across different analytic strategies.0
356005742022The Effect of Spontaneous LH Surges on Pregnancy Outcomes in Patients Undergoing Letrozole-HMG IUI: A Retrospective Analysis of 6,285 Cycles.0
355982092022Characterization and complete genome analysis of a novel Escherichia phage, vB_EcoM-RPN242.0
355809842022Tmax profile in computed tomography perfusion-based RAPID software maps influences outcome after mechanical thrombectomy in patients with basilar artery occlusion.0
355765212022Extension of the Alternative Intravenous Dosing Regimens of Atezolizumab into Combination Settings through Modeling and Simulation.0
355697462022Implantation of acellular dermal matrix to prevent frontotemporal depression following minipterional craniotomy for the surgical clipping of unruptured intracranial aneurysms.0
355433362022Prognostic significance of serum potassium in patients hospitalized for acute heart failure.0
355353002022Cost-Effectiveness and Budget Impact Analyses of Tisagenlecleucel in Pediatric and Young Adult Patients with Relapsed or Refractory B-Cell Acute Lymphoblastic Leukemia from the Singapore Healthcare System Perspective.0
355238602022Therapeutic response in the HAWK and HARRIER trials using deep learning in retinal fluid volume and compartment analysis.0
355235852022Positive Airway Pressure and Cognitive Disorders in Adults With Obstructive Sleep Apnea: A Systematic Review of the Literature.0
355134422022Hypertension treatment cascade in India: results from National Noncommunicable Disease Monitoring Survey.0
355020632022Triamcinolone acetonide injection in the treatment of upper eyelid retraction in Graves' ophthalmopathy evaluated by 3.0 Tesla magnetic resonance imaging.0
355019242022A high-throughput visual screening method for p-hydroxybenzoate hydroxylase to increase phenolic compounds biosynthesis.0
354867432022In vivo two-photon fluorescence lifetime imaging microendoscopy based on fiber-bundle.0
354837942022Time Spent Engaging in Health Care Among Patients With Left Ventricular Assist Devices.0
354439672022Implementation of virtual reality technology to decrease patients' pain and nervousness during colonoscopies: a prospective randomised controlled single-blinded trial.0
354057182022Volumetric Analysis of Intravoxel Incoherent Motion Diffusion-Weighted Imaging for Predicting the Response to Chemotherapy in Patients With Locally Advanced Non-Small Cell Lung Cancer.0
354050882022De-escalated neoadjuvant pertuzumab plus trastuzumab therapy with or without weekly paclitaxel in HER2-positive, hormone receptor-negative, early breast cancer (WSG-ADAPT-HER2+/HR-): survival outcomes from a multicentre, open-label, randomised, phase 2 trial.0
353976642022Population pharmacokinetics of rucaparib in patients with advanced ovarian cancer or other solid tumors.0
353849082022Comprehensive Review of Cardiovascular Complications of Coronavirus Disease 2019 and Beneficial Treatments.0
353345022022Exchange Bias in Molecule/Fe3 GeTe2 van der Waals Heterostructures via Spinterface Effects.0
352595572022Water clarity mapping of global lakes using a novel hybrid deep-learning-based recurrent model with Landsat OLI images.0
351682032022The prognostic value of the lymph node ratio in patients with distal cholangiocarcinoma after curative intended surgery: A single-center retrospective study.0
351334852022A nomogram strategy for identifying the subclassification of IDH mutation and ATRX expression loss in lower-grade gliomas.0
350932552022The hidden mortality of pediatric firearm violence.1
350814412022Palliative Care Exposure Relative to Predicted Risk of Six-Month Mortality in Hospitalized Adults.0
347554382022Who's slipping through the cracks? A comprehensive individual, clinical and health system characterization of people with virological failure on first-line HIV treatment in Uganda and South Africa.0
346775712022The effect of mental health conditions on the use of oral anticoagulation therapy in patients with atrial fibrillation: the FinACAF study.0
330653112022Rates of Intestinal Resection and Colectomy in Inflammatory Bowel Disease Patients After Initiation of Biologics: A Cohort Study.7
324494192022Comparison of two management options for labor induction following unsuccessful prostaglandin E2 ripening.0
357305162022Letter to the Editor: Rethinking The Cost Of Antipsychotic Treatment: The Average Cost Of The Drugs Used In Turkey In 2020.0
357239582022The Impact of Care Bundles on the Incidence of Surgical Site Infections: A Systematic Review.0
357185142022[Comparison of chloroplast genomes of medicinal plants in Aristolochiaceae].0
357159302022Description of a European memory clinic cohort undergoing amyloid-PET: The AMYPAD Diagnostic and Patient Management Study.0
357133792022Multicenter Repeatability and Reproducibility of MR Fingerprinting in Phantoms and in Prostatic Tissue.0
357125372022Complete mitochondrial genome of the Kyrghyz racerunner (Eremias nikolskii Bedriaga, 1905) from Kyrgyzstan.0
357059012022Frequency and predictors of unspecific medical diagnoses in the emergency department: a prospective observational study.0
357009712022Mirror-Image Comparison of Maintenance Electroconvulsive Treatment Effectiveness in Affective and Psychotic Disorders.0
356966262022Awareness of Colorectal Cancer Risk Factors in Palestine: Where Do We Stand?0
356782462022Impact of chronic total occlusion in a non-infarct-related coronary artery on contrast-associated nephropathy in acute ST-elevation myocardial infarction.0
356685752022Impact of the novel CYP2C:TG haplotype and CYP2B6 variants on sertraline exposure in a large patient population.0
356683622022Whole-genome-sequence-based characterization of an NDM-5-producing uropathogenic Escherichia coli EC1390.0
356678352022Automated Determination of Left Ventricular Function Using Electrocardiogram Data in Patients on Maintenance Hemodialysis.0
355804942022Efficient removal of trifluoroacetic acid from water using surface-modified activated carbon and electro-assisted desorption.0
355007132022Attributed radiative forcing of air pollutants from biomass and fossil burning emissions.0
354723642022Molecular cloning, expression in Escherichia coli and structural-functional analysis of a pyruvate kinase from Pyrobaculum calidifontis.0
354354232022Anesthetic gas consumption with target-controlled administration versus a semi-closed circle system with automatic end-tidal concentration control in an artificial lung model.0

Bibliography

Pubmed IDLast YearTitleAuthors
113696582001Cytogenetic analysis and clinical significance in adult T-cell leukemia/lymphoma: a study of 50 cases from the human T-cell leukemia virus type-1 endemic area, Nagasaki.Itoyama T et al
119988932002Genetic instability of adult T-cell leukemia/lymphoma by comparative genomic hybridization analysis.Tsukasaki K et al
150271212004Identifying progression-associated genes in adult T-cell leukemia/lymphoma by using oligonucleotide microarrays.Tsukasaki K et al

Citation

Gianluigi Castoldi ; Antonio Cuneo

Adult T-cell leukemia/lymphoma (ATLL)

Atlas Genet Cytogenet Oncol Haematol. 2005-05-01

Online version: http://atlasgeneticsoncology.org/haematological/2032/adulttleuklymphid2032

External Links