Leucèmia limfàtica crònica atípica : concepte i ús

La leucèmia limfàtica crònica (LLC) és un càncer de la sang caracteritzat per l'acumulació de limfòcits B madurs, un tipus de glòbuls blancs que, en condicions normals, junt als limfòcits T, s'encarreguen de combatre les infeccions. La leucèmia limfàtica crònica atípica és una variant de la LLC amb diferències citogenètiques i fenotípiques, que avui en dia no té una definició fenotípica formal i freqüentment se'n fa un ús equívoc. Aquest article defensa que, tot i que de moment una distinció diagnòstica no té implicacions terapèutiques, és important establir una definició precisa de la LLC atípica perquè això pot canviar properament.La leucemia linfática crónica (LLC) es un cáncer de la sangre caracterizado por la acumulación de linfocitos B maduros, un tipo de glóbulos blancos que, en condiciones normales, junto a los linfocitos T, se encargan de combatir las infecciones. La leucemia linfática crónica atípica es una variante de la LLC con diferencias citogenéticas y fenotípicas, que hoy en día no tiene una definición fenotípica formal y frecuentemente se utiliza equívocamente. Este artículo defiende que, aunque actualmente una distinción diagnóstica no tiene implicaciones terapéuticas, es importante establecer una definición precisa de la LLC atípica porque podría cambiar en el futuro próximo.Chronic lymphocytic leukemia (CLL) is a type of cancer characterized by the accumulation of mature B lymphocytes, a type of white blood cell that, under normal conditions, together with T lymphocytes, are responsible for combating infections. Atypical chronic lymphocytic leukemia is a CLL variant with cytogenetic and phenotypic differences that has no formal phenotypic definition and is used frequently in an equivocal way. This paper defends that, even though this diagnostic distinction has no therapeutic implications, it is important to establish a precise definition of atypical CLL because this may change in the near future

Detecció simptomàtica vs detecció asimptomàtica de la recaiguda en el limfoma fol·licular

El limfoma fol·licular és un subtipus de limfoma sense cura però l'esperança de vida és llarga. De fet, és habitual fer proves d'imatge de seguiment en pacients que es troben en remissió després d'haver rebut tractament. Aquest article té com a objectiu valorar si realment aquestes proves de seguiment tenen utilitat ja que provoquen efectes perjudicials. Per fer-ho, es va comparar la supervivència lliure de malaltia entre pacients amb recaiguda asimptomàtica i simptomàtica. La conclusió és que hi ha una aparent major supervivència lliure de malaltia de pacients amb recaiguda asimptomàtica degut al perfil més indolent de les recaigudes en el grup. Tanmateix, assenyalen un disseny de l'estudi incapaç de respondre a l'objectiu principal deixant així una porta oberta a futures investigacions.El linfoma folicular es un subtipo de linfoma sin cura pero la esperanza de vida es larga. De hecho, es habitual hacer pruebas de imagen de seguimiento en pacientes que se encuentran en remisión después de haber recibido tratamiento. Este artículo tiene como objetivo valorar si realmente estas pruebas de seguimiento tienen utilidad ya que provocan efectos perjudiciales. Para ello, se comparó la supervivencia libre de enfermedad entre pacientes con recaída asintomática y sintomática. La conclusión es que hay una aparente mayor supervivencia libre de enfermedad de pacientes con recaída asintomática debido al perfil más indolente de las recaídas en el grupo. Sin embargo, señalan un diseño del estudio incapaz de responder al objetivo principal dejando una puerta abierta a futuras investigaciones.Follicular lymphoma (FL) is a common subtype of indolent (generally incurable) lymphoma but patients have a long life expectancy. In fact, it is standard practice to offer routine surveillance imaging to patents in remission after therapy. The goal of this study was to assess whether routine surveillance is useful, considering its well-known side effects. We compared the survival of patients with FL in whom relapse was detected by routine surveillance computed tomography (CT) with that of those suffering a symptomatic relapse. The authors concluded that the apparent longer progression-free survival among patients with asymptomatic relapse was due to lower risk relapses in this group. However, it is important to realize that the design of this study could not aim to answer the main question, leaving an open door to further studies

Incidència de malaltia tromboembòlica en pacients amb síndromes limfoproliferatives tractades amb lenalidomida

La lenalidomida és un fàrmac que habitualment s'utilitza per tractar el mieloma, un càncer de les cèl·lules plasmàtiques. Un dels seus efectes secundaris és la malaltia tromboembòlica (MTE), una formació d'un coàgul en el sistema venós. Recentment, la lenalidomida s'ha utilitzat amb èxit pel tractament d'algunes síndromes limfoproliferatives però no queda clar si també augmenta el risc de MTE en aquests pacients. Investigadors del departament d'Hematologia de l'Hospital Germans Trias i Pujol - UAB han dut a terme una revisió sistemàtica de la incidència de la MTE en pacients amb síndromes limfoproliferatives tractades amb lenalidomida per estudiar quin és el risc trombòtic conferit.La lenalidomida es un fármaco que habitualmente se utiliza para tratar el mieloma, un cáncer de células plasmáticas. Uno de sus efectos secundarios es la enfermedad tromboembólica (MTE), una formación de un coágulo en el sistema venoso. Recientemente, la lenalidomida se ha utilizado con éxito para el tratamiento de algunos síndromes limfoproliferativos pero no queda claro si también aumenta el riesgo de MTE en estos pacientes. Investigadores del departamento de Hematología del Hospital Germans Trias i Pujol - UAB han llevado a cabo una revisión sistemática de la incidencia de la MTE en pacientes con síndromes limfoproliferativos tratados con lenalidomida parar estudiar cual es el riesgo trombótico conferido. Incidencia de enfermedad tromboembólica en pacientes con síndromes linfoproliferativos tratados con lenalidomidaLenalidomide is a drug commonly used to treat myeloma, a cancer of plasma cells. One of its side effects is venous thromboembolism (VTE), which is the development of a blood clot in the veins. Most recently, lenalidomide has been used to treat lymphoproliferative disorders but it remains unclear whether the risk of VTE is also increased in this patients. Researchers from the Department of Hematology of the Germans Trias i Pujol Hospital - UAB carried out a systematic review of the incidence of VTE in patients with lymphoproliferative disorders treated with lenalidomide to study which is the thrombotic risk conferred

La importància del grau histològic en el limfoma folicular

El limfoma fol·licular (LF) es divideix en 3 graus histològics diferents. Sembla existir una relació entre aquests graus i alguns subtipus del limfoma difús de les cèl·lules grans B (LDCGB), en forma de continu biològic, amb el LF de grau 3 (LF3) com a intermediari. Malgrat que aquesta relació no està ben establerta encara, normalment es recomana el mateix tractament pel LDCGB i el LF3. En una carta a l'editor de la revista Annals of Oncology, investigadors de la UAB plantegen quees desenvolupin estudis que analitzessin especificament el LF3. A més, atès que el tractament pel LDCGB probablement canviï en els propers anys, caldria investigar si aquest també hauria de canviar per LF3, i en quina direcció hauria de fer-ho.El linfoma folicular (LF) se divide en 3 grados histológicos distintos. Parece existir una relación entre estos grados y algunos subtipos del linfoma difuso de células grandes B(LDCGB), en forma de contínuo biológico, con el LF de grado 3 (LF3) como intermediario. Aunque esta relación no está bien establecida aún, normalmente se recomienda el mismo tratamiento para el LDCGB y el LF3. En una carta al editor de la revista Annals of Oncology, investigadores de la UAB plantean que se desarrollen estudios que analicen LF3 de forma específica. Además, dado que el tratamiento para el LDCGB probablemente cambie en los próximos años, cabría investigar si este también debería cambiar para LF3, y en qué dirección debería hacerlo.Folicular Lymphoma (FL) is divided into 3 different grades. It seems that there is a relationship between the lower grades and some subtypes of diffuse large B-cell lymphoma (DLBCL), in the form of a biological continuum, with the grade 3 FL (FL3) as an intermediary. Although this relationship remains unclear, it is normal to use the same treatment for DLBCL and FL3. In a letter to the editor of Annals of Oncology, researchers from de AUB pose that specific studies about LF3 should be developed. Besides, since the treatment used to DLBCL will probably change in the next years, it will be necessary to change LF3's treatment and to look for the best possible treatmen

Estimating the Lifetime Treatment Burden of Patients With Follicular Lymphoma: A Retrospective Study Using Real-World Multicenter Data

PURPOSE Although follicular lymphoma is characterized by long natural history and frequent relapses, data on the number of patients receiving subsequent therapy lines are scarce. To perform reliable health economical calculations for various treatment options, data regarding the lifetime number of therapy courses are needed. The purpose of this study was to use real-world data to create a model that could estimate the treatment burden over a 20-year period. MATERIALS AND METHODS We performed a 20-year simulation on the basis of retrospectively obtained multicenter data of 743 patients with follicular lymphoma. The simulation was carried out in two steps: First, a competing risk model on the basis of Weibull distribution was used to simulate the state transitions from diagnosis onward and from first-line therapy onward. Then, the data were completed by imputing on the basis of the existing data. Completion of data was repeated for 1,000 times to estimate reliability. RESULTS In 20 years, 97% (2.5-97.5 percentile range: 96%-98%), 66% (61%-70%), 34% (30%-41%), and 15% (9%-18%) of the patients received first-line, second-line, third-line, and fourth-line therapies, respectively. The median number of therapy lines received by each patient was two. CONCLUSION Despite long remissions, approximately two thirds of the patients receive at least two lines and one-third at least three lines of therapy during their lifetime

Female patients with follicular lymphoma have a better prognosis if primary remission lasts over 24 months

Findings regarding the role of sex in follicular lymphoma (FL) are contradictory and the prognostic value of sex among patients with early progression of disease (POD) remains unclear. We collected real-life data from nine hospitals in Finland and Spain including 1020 FL patients to study the influence of sex on disease outcome. The median follow-up duration was 67 months (range 0-226 months). Female patients showed better progression-free survival (PFS) (hazard ratio [HR], 0.720; 95% confidence interval [CI], 0.588-0.881), disease-specific survival (DSS) (HR, 0.653; 95% CI, 0.448-0.951), and overall survival (OS) (HR, 0.653; 95% CI, 0.501-0.853) than male patients. However, there were no significant sex differences in prognosis in patients with early POD. This study strengthens the understanding that male sex is an adverse prognostic factor for FL. However, this difference does not apply to patients with early POD.Peer reviewe

Divergent leukaemia subclones as cellular models for testing vulnerabilities associated with gains in chromosomes 7, 8 or 18

Haematopoietic malignancies are frequently characterized by karyotypic abnormalities. The development of targeted drugs has been pioneered with compounds against gene products of fusion genes caused by chromosomal translocations. While polysomies are equally frequent as translocations, for many of them we are lacking therapeutic approaches aimed at synthetic lethality. Here, we report two new cell lines, named MBU-7 and MBU-8, that differ in complete trisomy of chromosome18, a partial trisomy of chromosome 7 and a tetrasomy of the p-arm of chromosome 8, but otherwise share the same mutational pattern and complex karyotype. Both cell lines are divergent clones of U-937 cells and have the morphology and immunoprofile of monocytic cells. The distinct karyotypic differences between MBU-7 and MBU-8 are associated with a difference in the specific response to nucleoside analogues. Taken together, we propose the MBU-7 and MBU-8 cell lines described here as suitable in vitro models for screening and testing vulnerabilities that are associated with the disease-relevant polysomies of chromosome 7, 8 and 18

Survival of patients with mantle cell lymphoma in the rituximab era : Retrospective binational analysis between 2000 and 2020

Mantle cell lymphoma (MCL) is a rare peripheral B-cell lymphoma characterised by eventual relapse and progression towards a more aggressive disease biology. With the introduction of rituximab- and cytarabine-based immunochemotherapy regimens, the prognosis of the disease has changed dramatically over the last two decades. To assess the real-world survival of patients with MCL, we used a population-based cohort of 564 patients with MCL who were diagnosed and treated between 2000 and 2020. Patient data were collected from seven Finnish treatment centres and one Spanish treatment centre. For the entire patient population, we report a 2-year overall survival (OS) rate of 77%, a 5-year OS of 58%, and a 10-year OS of 32%. The estimated median OS was 80 months after diagnosis. MCL is associated with increased mortality across the entire patient population. Additionally, we assessed the survival of patients after MCL relapse with the aim of establishing a cut-off point of prognostic significance. Based on our statistical analysis of survival after the first relapse, disease progression within 24 months of the initial diagnosis should be considered as a strong indicator of poor prognosis.publishedVersionPeer reviewe