Analyzing the incidence, survival, and demographic trends of B-cell prolymphocytic leukemia

Background: B-cell prolymphocytic leukemia (B-PLL) is a rare disease, consisting <1% of mature B-cell malignancies. B-PLL is often refractory to chemotherapy, with a median survival of 3 years. Due to its rarity, no large cohort studies exist elucidating outcomes. Methods: All B-PLL patients >15 years were identified in the National Cancer Institute’s Surveillance, Epidemiology, and End Results (SEER) database between 2000-2020. Statistical analysis explored demographic variables like age; categorized as adolescent or young adult (AYA) and adults. In adults, differences in survival due to factors such as sex, race/ethnicity, household income, rurality, and age categorized in 10-15 year buckets was analyzed. Results: B-PLL patients were predominantly white (78%), over 40 years (96%) and mostly residing in metropolitan areas (90%). Interestingly, the AYA cohort were mostly female (70%). 35% of the AYA patients were Hispanic, while being only 9% in adults. Among adults, the rate of Asian/Pacific-Islander patients that were alive at the time of the data query was 53% compared to 34% in Hispanic, 31% in black, and 24% in white patients (p=0.025). Younger age was also associated with greater chances of survival (p<0.001). Conclusions: In line with known poor prognosis of the disease, 23% patients were alive at the time of data query. Female and Hispanic patients were overrepresented in the AYA age group. In the adult group Asian/Pacific-Islander patients had better survival outcomes, as did younger patients. Further research is necessary to explore why B-PLL incidence in AYA patients is higher among Hispanic and females.

Comparative analysis of leukemia and risk estimation in working age population between provinces of Ecuador / Análisis comparativo de leucemia y estimación del riesgo en la población en edad laboral entre provincias del Ecuador

Leukemia is associated with exposure to radiation, benzene derivatives, and pesticides. Previous research has documented an increase in work-related leukemia in the Latin American Andean region. To date, there are only few studies in Ecuador on the impact of oil exploitation on adjacent indigenous communities. Our study aims to show the impact of leukemia on the working-age population. For the calculation of morbidity and mortality rates, we used hospital discharge and death records from the National Institute of Statistics of Ecuador. These data were collected and adjusted to the corresponding province's population for further analysis. Large differences were observed between provinces in adjusted rates of leukemia mortality and morbidity in the working-age population. The variations in altitude among different areas in Ecuador give the provinces a distinct geographic identity. Likewise, the provinces with the highest morbidity and mortality rankings, such as Azuay, Loja, Imbabura, and Tungurahua, have an average altitude above 2000 meters. As a result, there are variations in the average temperature, exposure to solar and cosmic radiation, and mining and farming methods. The observed differences warrant the future collection of geolocation data for affected individuals. This could help to better understand how leukemia cases have demogrpahic hotspots in the country, identify possible risk factors associated with the disease in each region, and design more effective prevention and control strategies.

La leucemia es una enfermedad a consecuencia, además de factores genéticos, de la exposición a radiaciones, derivados del benceno y pesticidas. Investigaciones anteriores han documentado un aumento de la leucemia ocupacional en la región andina de América Latina. Hasta la fecha, existen sólo unos pocos estudios en Ecuador sobre el impacto de la explotación petrolera en las comunidades indígenas. Nuestro objetivo es mostrar el impacto de la leucemia en la población en edad de trabajar. Para el cálculo de las tasas de morbimortalidad se utilizaron los registros de altas hospitalarias y defunciones del Instituto Nacional de Estadística del Ecuador. Estos datos fueron recopilados y estimadas las tasas ajustadas. Se observaron grandes diferencias entre provincias en las tasas ajustadas de mortalidad y morbilidad por leucemia en la población en edad de trabajar. Asimismo, las provincias con mayor ranking de morbilidad y mortalidad, como Azuay, Loja, Imbabura y Tungurahua, coinciden en tener una altitud promedio superior a los 2000 metros. Hay provincias de baja altitud en la costa y provincias por encima de los 2000 metros en la sierra, lo que le da a las provincias del Ecuador una identidad geográfica distintiva. Como resultado, existen variaciones en la temperatura promedio, la exposición a la radiación solar y cósmica, y actividades de minería y agricultura. Las diferencias observadas, recomiendan la recopilación futura de datos de geolocalización de las personas afectadas. Esto podría ayudarnos a comprender mejor cómo se distribuyen los casos de leucemia, identificar posibles factores de riesgo asociados a la enfermedad en cada región y diseñar estrategias de prevención y control más efectivas.

Disseminated juvenile xanthogranuloma secondary to chemotherapeutic treatment of acute lymphoblastic leukemia

Juvenile xanthogranuloma is a benign, reactive, self-limiting disorder within the non-Langerhans cell histiocytosis group. It primarily affects infants and preschoolers, and occasionally in adults. Some cases report concurrent non-LCH and leukemia, with bone marrow being the second common pathology. We present a case of JXG with acute lymphoblastic leukemia in a 17-year-old male. Emphasizing the importance of considering the possibility of apparently disparate disorders in a patient, especially with unusual clinical findings.

Sangrado uterino anormal como presentación inicial de malignidades hematológicas / Abnormal uterine bleeding as the initial presentation of hematologic malignancies

El sangrado uterino anormal tiene una etiología variable, que va desde causas estructurales hasta causas funcionales, que se describen clásicamente en el acrónimo PALM-COEIN. No obstante, hay una pobre sensibilización de este síntoma como un marcador de enfermedades graves. En esta revisión se describe la relación de la hemorragia uterina anormal como síntoma clave o de presentación de malignidad hematológica, así como la posible relación con la hemofilia adquirida secundaria a neoplasia hematológica como causal del evento hemostático. Se realizó búsqueda en la literatura, con la mayoría de los artículos obtenidos de Medline, 24 de los cuales cumplieron con los objetivos para resolver la pregunta de investigación. Se encontraron diferentes malignidades hematológicas asociadas a sangrado uterino anormal, de las cuales la hemofilia adquirida y la trombocitopenia como potenciales causales de esta; la mayor correlación fue con leucemia, seguido de linfomas, y en menor cuantía la asociación con mieloma múltiple.

Abnormal uterine bleeding has a variable etiology, ranging from structural to functional causes, classically described by the acronym PALM-COEIN. However, there is poor awareness of this symptom as a marker of serious disease; in this review, we describe the relationship of abnormal uterine bleeding as a key symptom or debut of hematologic malignancy, as well as its possible relationship to acquired hemophilia secondary to hematologic neoplasia as causative of the hemostatic event. A literature search was performed, with most of the articles obtained from Medline, 24 of which met the objectives to solve the research question. Different hematological malignancies associated with abnormal uterine bleeding were found, of which acquired hemophilia and thrombocytopenia were found as potential causes; the highest correlation was with leukemia, followed by lymphomas, and to a lesser extent the association with multiple myeloma.

Resultados de dos protocolos de tratamiento para la leucemia linfoide aguda infantil en un hospital peruano / Results of two treatment protocols for children's acute lymphoid leukemia in a Peruvian hospital

Resumen Introducción. La leucemia linfoide aguda (LLA) es la neoplasia infantil más frecuente. Existen diversos protocolos de tratamiento, por lo que resulta importarte conocer su impacto en la supervivencia. Materiales y métodos. Estudio comparativo, cohorte retrospectiva. Se incluyeron pacientes menores de 16 años con diagnóstico de LLA tratados con protocolo ALL IC-BFM 2009 o esquema LLA 2008 durante enero 2020 a noviembre 2023. Se revisaron historias clínicas de los pacientes. Como instrumento se tuvo una ficha de recolección de datos. Se empleó el análisis de supervivencia de Kaplan Meier para determinar la sobrevida global (SG) y supervivencia libre de enfermedad (SLE). Resultados. Se incluyeron 107 pacientes. El grupo etario de 2-9 años fue el más prevalente en ambos grupos (57.1% y 63.3%). La SG a 32.2 meses fue 73.6% con el protocolo ALL IC BFM 09 y 43.5% con el esquema LLA 2008 (p=0.03). La SLE a 30.8 meses fue 82.3% y 51.8% respectivamente (p=0.04). Discusión. En nuestro estudio, la SG y SLE fue mayor al 70% para el protocolo ALL IC BFM 09, siendo estos hallazgos similares a lo reportado por literatura internacional. La supervivencia con el protocolo ALL IC BFM 2009 fue superior al esquema LLA 2008.

Abstract Introduction. Acute lymphoid leukemia is the most common childhood neoplasm. There are various treatment protocols, so it is important to know their impact on survival. Material and methods. Comparative study, retrospective cohort. Patients under 16 years of age with a diagnosis of ALL treated with the ALL IC-BFM 2009 protocol or the LLA 2008 scheme were included during January 2020 to November 2023. The patients' medical records were reviewed, and a data collection sheet was used as an instrument. Kaplan Meier survival analysis was used to determine overall survival (OS) and disease-free survival (DFS). Results. 107 patients were included. The age group of 2-9 years was the most prevalent in both groups (57.1% and 63.3%). OS at 32.2 months was 73.6% with the ALL IC BFM 2009 protocol and 43.5% with the LLA 2008 scheme (p=0.03). The DFS at 30.8 months was 82.3% and 51.8% respectively (p=0.04). Discussion. In our study, OS and DFS were greater than 70% for the ALL IC BFM 2009 protocol, these findings being similar to those reported in international literature. Survival with the ALL IC BFM 2009 protocol was superior to the previous institutional protocol.

Periodontal status in adult patients with leukemia: a literature review / Condição periodontal em pacientes adultos com leucemia: revisão de literatura

Periodontal disease is a highly prevalent chronic inflammatory disease that affects the tissues that support the teeth, while leukemia is a type of malignous cancer that affects the production of blood cells. Recent studies suggest that immune response and microbial disbiosis related to periodontal disease may be associated with an increased risk of developing leukemia and may affect its prognosis, as well as leukemia type and treatment may also have effects on the periodontium, demanding a interdiscipinary approach of these patients. The aim of this study was to conduct a literature review to assess the association between periodontal disease and leukemia in adult patients. An electronic database serch using the descriptors was performed. Clinical studies with periodontal examination in adult individuals with leukemia were selected. After literature search, 9 studies were reviewed. Gingival bleeding and periodontal pockets were frequent findings. Periodontitis prevalence varied among studies, ranging from 29% to 82,4% in patients diagnosed with leukemia. The relationship between periodontal disease and leukemia is complex and multifaceted and there are few studies available in adults, with heterogeneous exam protocols. Still, the high prevalence of gingivitis and periodontitis found in the studies suggest that periodontal diagnosis and treatment could be a helpful tool to prevent further complications in leukemia treatment.

A doença periodontal é uma doença inflamatória crônica altamente prevalente e que afeta os tecidos que sustentam os dentes, enquanto a leucemia é um tipo de câncer maligno que afeta a produção de células sanguíneas. Estudos recentes sugerem que a resposta imune e a disbiose microbiana relacionada a doença periodontal podem estar associadas a um risco aumentado de desenvolver leucemia e pode afetar o prognóstico da doença, assim como o tipo de leucemia e o tratamento também podem ter efeitos no periodonto, exigindo uma abordagem interdisciplinar desses pacientes. O objetivo deste estudo foi realizar uma revisão de literatura para avaliar a associação entre doença periodontal e leucemia em pacientes adultos. Foi realizada uma busca eletrônica em bancos de dados utilizando os descritores. Foram selecionados estudos clínicos com exame periodontal em indivíduos adultos com leucemia. Após busca na literatura, 9 estudos foram revisados. Sangramento gengival e bolsas periodontais foram achados frequentes. A prevalência da periodontite variou entre os estudos, sendo de 29% a 82,4% em pacientes diagnosticados com leucemia. A relação entre doença periodontal e leucemia é complexa e multifacetada e existem poucos estudos disponíveis em adultos, com protocolos de exames heterogêneos. Ainda assim, a alta prevalência de gengivite e periodontite encontrada nos estudos sugere que o diagnóstico e o tratamento periodontal podem ser uma ferramenta útil para prevenir maiores complicações no tratamento da leucemia.

Priapismo y leucemia mieloide crónica en adolescente. Debut poco frecuente. Reporte de caso / Priapism and chronic myeloid leukemia in an adolescent. Rare debut presentation. A case report

El priapismo es una erección dolorosa y persistente acompañada o no de estímulo sexual. Una causa poco frecuente de esta anormalidad es la leucemia mieloide crónica. Se han reportado pocos casos de priapismo como manifestación inicial de una leucemia de este tipo en pacientes adolescentes. A continuación, se informa el caso de un paciente de 16 años de edad que presentó priapismo como manifestación inicial de una leucemia mieloide crónica. Durante su evolución, no se realizó aspiración de los cuerpos cavernosos. Se inició tratamiento hematológico específico y, ante la persistencia del priapismo, fue necesario realizar un shunt de cuerpos cavernosos en dos ocasiones, tratamiento a pesar del cual existen altas probabilidades de secuelas.

Priapism is a painful and persistent erection, with or without sexual stimulation. A rare cause of such abnormality is chronic myeloid leukemia. Few cases of priapism as an initial manifestation of this type of leukemia have been reported in adolescent patients. Here we describe the case of a 16-year-old patient who presented with priapism as the initial manifestation of chronic myeloid leukemia. No cavernosal aspiration was performed. A specific hematological treatment was started and, given the persistence of priapism, the patient required 2 corpora cavernosa shunt procedures; despite this treatment, there is a high probability of sequelae.

Mycobacterium tuberculosis y virus linfotrópico humano, una co-infección fatal / Mycobacterium tuberculosis and human lymphotropic virus, a fatal co-infection

La tuberculosis es una infección de alta incidencia en Latinoamérica. Su presentación como infección activa está determinada por factores de riesgo del hospedero. Comunicamos el caso clínico de una mujer joven que presentó una forma grave de tuberculosis pulmonar. Al explorar sus factores de riesgo se confirmó un estado de inmunosupresión profundo, causado por un linfoma de células T, asociada a una co-infección por virus linfotrópico T humano tipo 1. Se destacan los aspectos microbiológicos y de pronóstico de la co-infección de Mycobacterium tuberculosis y HTLV-1

Tuberculosis is a high-incidence infection in Latin America. Its presentation as an active infection is determined by risk factors in the host. We report the case of a young woman who presented a severe form of pulmonary tuberculosis. When exploring her risk factors, a profound state of immunosuppression was found, caused by T-cell lymphoma, associated with co-infection with human lymphotropic virus. Microbiological and prognostic aspects of Mycobacterium tuberculosis and HTLV-1 co-infection are highlighted.

Therapeutic Choices in Patients with Ph-Positive Chronic Myelogenous Leukemia In Mexico in the Era of Tyrosine Kinase Inhibitors: Stem Cell Transplantation or Tyrosine Kinase Inhibitors? Fifteen Years Later

ABSTRACT Background: Chronic myelogenous leukemia is a neoplastic proliferation of the granulocytic series. In Mexico, chronic myelogenous leukemia accounts for approximately 10% of all leukemias. Tyrosine-kinase inhibitors are considered front-line therapy in high-income countries, whereas allogeneic hematopoietic stem cell transplantation is a recognized therapeutic approach, mainly in low- and middle-income countries. Objective: To analyze the overall survival of persons with chronic myelogenous leukemia who have received tyrosine-kinase inhibitors or allogeneic hematopoietic stem cell transplantation in a medical center, since 1994, and briefly discuss the current indications of these treatments in the tyrosine-kinase inhibitors era. Methods: We retrospectively analyzed all patients with a diagnosis of chronic myelogenous leukemia treated in a medical center between 1994 and 2023; subsets of individuals who received an allogeneic hematopoietic stem cell transplantation or tyrosine-kinase inhibitors therapy as first-line treatment were analyzed. Results: 60 persons with chronic myelogenous leukemia were treated with allogeneic hematopoietic stem cell transplantation or tyrosine-kinase inhibitors: 35 received an allogeneic hematopoietic stem cell transplantation, whereas 25 were given tyrosine-kinase inhibitors. All patients who underwent an allogeneic hematopoietic stem cell transplantation engrafted successfully, and the procedure was completed on an outpatient basis in most cases (29/35). The median survival in allogeneic hematopoietic stem cell transplantation was 78.3 months (CI 95%: 0-205.6) and in persons given tyrosine-kinase inhibitors the median was not reached. Conclusion: Tyrosine-kinase inhibitors were significantly superior to allogeneic hematopoietic stem cell transplantation in prolonging the overall survival of persons with chronic myelogenous leukemia in our single institution experience. (Rev Invest Clin. 2024;76(2):91-6)

Study of automated hematology analyzer抯 scatterplot patterns in white blood cell disorders

Background: Automated hematology analyzers produce scattergrams that can be used as screening tool for various hematological conditions and efficiently shorten turnaround times. Aim was to study scattergram patterns of various while blood cell disorders and assess their efficacy compared to a peripheral blood smear for diagnosis of various disorders. Methods: Scattergram findings generated by UniCel� DxH 800 automated hematology analyzer, a 5-part differential analyzer. The graphic displays have been compiled over a period of 3 months from blood samples received for CBC. Samples that the counter flagged as abnormal for white blood cell were chosen. Based on the scatterplots, a preliminary diagnosis was formed. It was compared with the peripheral blood smear (PBS) findings which were taken as the gold standard. Results: The scatterplots showed unique patterns for various disorders on the basis of location, shape, size, density of the cells and their clustering. The scattergram analyser showed 90% sensitivity and 88% specificity for diagnosing hematological disorders. A 97-100% accuracy rate was reported showing excellent correlation between PBS result and WBC parameter result in cell counter analyzers. Conclusions: Not all cases of haematological malignancy exhibit cytopenias or cytosis at initial presentation. Therefore, these scatter plots offer helpful information that prompts a hematopathologist to suspiciously screen the peripheral smear in cases with normal counts. Scattergram analysis suspects a diagnosis earlier than peripheral smear examination. Given their strong correlation with a variety of WBC disorders and confirmed by PBS, WBC scatterplots can be used as a screening tool.

Clinical outcome and prognosis of patients with acute myeloid leukemia submitted to chemotherapy with 5 years of follow-up

Abstract Objective The purpose of this study was to evaluate the clinical-epidemiological profile, associated risk factors and clinical outcomes of patients with acute myeloid leukemia (AML), identifying the main causes of morbidity and mortality and overall survival rate of patients at five years of follow-up. Method This was a retrospective cohort study evaluating the prognosis and clinical outcomes of 222 patients diagnosed with AML at three large hematology centers in Ceará (northeastern Brazil) over a period of five years. Results The mean age at diagnosis was 44.1 ± 16 years, with a female prevalence of 1.3:1. No additional relevant risk factors associated with the development of AML were found, except for the well-established cytogenetic assessment. The overall 5-year survival rate was 39.4% (95%CI: 35.47 - 42.17). The main causes of death were disease progression (37.72%; n = 84) and sepsis (31.58%; n = 70). Conclusion The clinical outcomes in our sample of AML patients were similar to those of other reported groups. Disease progression and infection were the main causes of death. Access to diagnostic flow cytometry and karyotyping was greater in our sample than in the national average. As expected, overall survival differed significantly according to the risk, as determined by cytogenetic testing.

Megakaryoblastic Acute Myeloid Leukemia Associated with a Testicular Tumour in a Young Adult: a Case Report / Leucemia Mieloide Aguda Megacarioblástica Associada a Tumor de Testículo em Adulto Jovem: um Relato de Caso

Acute megakaryoblastic leukemia (M7 AML) is a rare subtype of acute myeloid leukemia in adults, the incidence of which is higher in children aged 1 to 3 years, especially in patients with Down Syndrome; and in the age group between 60 and 70 years old, with an adverse prognosis. We report the case of a 28-year-old male patient, with a history of non-seminoma germ cell tumour of the testis, diagnosed with M7 AML. Nine months after performing an orchiectomy to remove the testicular tumour, the patient developed dyspnea, dry cough and asthenia, associated with the presence of erythematous-purple lesions on the skin, ascites and pleural effusion. The myelogram demonstrated medullary hypocellularity, with the presence of 53% of blastic, pleomorphic and bulky cells, with positivity for the markers CD34, CD31 and CD117 in immature cells in immunohistochemistry. Despite undergoing cycles of chemotherapy with cisplatin and a BEP regimen (Bleomycin, Etoposide and Cisplatin), the patient presented with chest tomography with the presence of pulmonary nodules and magnetic resonance imaging of the skull and neuraxial with infiltration of the bone marrow in the spine and cranial vault, resulting in with neurological impairment and died. In view of the case presented, we observed agreement with previous reports of the adverse prognosis of M7 AML in young adults and we questioned its relationship with germ cell tumour. (AU)

A leucemia megacarioblástica aguda (LMA M7) é um subtipo raro em adultos de leucemia mielóide aguda, cuja incidência é maior em crianças de 1 a 3 anos, especialmente em pacientes portadores de Síndrome de Down; e na faixa etária entre 60 e 70 anos, com um prognóstico adverso. Relatamos o caso de um paciente, do sexo masculino, 28 anos, com histórico de tumor germinativo não seminoma de testículo, diagnosticado com LMA M7. Nove meses após a realização de uma orquiectomia para a retirada do tumor testicular, o paciente apresentou quadro de dispneia, tosse seca e astenia, associado a presença de lesões eritemato-arroxeadas na pele, ascite e derrame pleural. O mielograma demonstrou hipocelularidade medular, com presença de 53% de células blásticas, pleomórficas e volumosas, com a positividade para os marcadores CD34, CD31 e CD117 em células imaturas na imunohistoquímica. Apesar da realização de ciclos de quimioterapia com cisplatina e esquema BEP (Bleomicina, Etoposídeo e Cisplatina), o paciente apresentou Tomografia de tórax com presença de nódulos pulmonares e ressonância magnética de crânio e neuroeixo com infiltração da medula óssea em coluna vertebral e calota craniana, intercorrendo com comprometimento neurológico e foi a óbito. Diante do caso apresentado observamos a concordância com relatos prévios do prognóstico adverso da LMA M7 em jovens adultos e indagamos a sua relação com o tumor de células germinativas. (AU)

Linfoma extranodal de epiglotis en un paciente con recaída de leucemia linfoide aguda de células T. Presentación de un caso / Extranodal lymphoma of the epiglottis in a patient with relapsed T-cell acute lymphoid leukemia. Case report

Los linfomas localizados en la laringe representan un porcentaje muy bajo dentro de los comprendidos en los tumores de cabeza y cuello en la edad pediátrica. El linfoma no Hodgkin es el subtipo más comúnmente reportado en la literatura, el cual dependiendo de su etiología y extensión determinará el pronóstico del paciente. La certeza del diagnóstico, que suele ser muy difícil de alcanzar, se confirma generalmente mediante una biopsia de tejido. En la actualidad, no hay reportes de la literatura acerca de linfomas leucemoides diseminados a laringe. Se presenta el caso de un paciente masculino adolescente de 17 años con diagnóstico de una leucemia linfoide aguda con recaída extra-nodal en la laringe por falla en el esquema quimioterapéutico instaurado.

Lymphomas located at the level of the larynx represent a very low percentage of head and neck tumors in the pediatric age group. Non-Hodgkin's lymphoma is the most reported subtype in the literature, which depending on its etiology and extension will determine the patient's prognosis. Diagnostic certainty, which is often very difficult to achieve, is usually confirmed by tissue biopsy. At present, there are no reports in the literature about leukemoid lymphomas disseminated to the larynx. We present the case of a 17-year-old adolescent male patient diagnosed with acute lymphoid leukemia with extranodal relapse in the larynx due to failure of the chemotherapeutic regimen.

Tratamiento de la recaída de pacientes pediátricos con leucemia linfoblástica aguda en la Argentina: resultados de un ensayo clínico y una cohorte prospectiva / Treatment of Relapse in Pediatric Patients with Acute Lymphoblastic Leukemia in Argentina: Results from a Clinical Trial and a Prospective Cohort

Introducción: desde 2002, el Grupo Argentino para el Tratamiento de la Leucemia Aguda (GATLA) implementa protocolos del grupo Berlín-Frankfurt-Münster (BFM) como tratamiento estándar de las recaídas de la leucemia linfoblástica aguda (LLA). En 2010, el BFM generó el protocolo IntReALL 10, que en la Argentina se implementó con las limitaciones propias de la región. Población y métodos: 180 pacientes menores de 18 años fueron tratados entre 2010 y 2015 por una LLA recaída de alto riesgo en la Argentina siguiendo un protocolo de recaída del BFM que comparó en forma abierta el tratamiento estándar con una terapéutica innovadora (experimental); esta incluyó el fármaco clofarabina. Se evaluaron 171 pacientes, de los cuales 78 pacientes fueron aleatorizados en forma centralizada (ensayo clínico) y 93 fueron asignados a una de las ramas según el criterio del grupo tratante (cohorte prospectiva). La cohorte donde la asignación del tratamiento no fue aleatorizada fue analizada realizando un ajuste por sexo, edad y por la presencia o no de síndrome de Down, cromosoma Philadelphia e inmunofenotipo T. Resultados: los pacientes que recibieron el tratamiento experimental tuvieron peores resultados (el doble de mortalidad a cinco años) que los que recibieron tratamiento estándar. Esta diferencia alcanzó significancia estadística tanto en el ensayo clínico (p=0,001) como en la cohorte prospectiva (p=0,0009). Conclusiones: nuestros resultados avalan continuar con la rama estándar de los protocolos tipo BFM para el tratamiento de las recaídas de la LLA y fueron concordantes con las conclusiones del grupo ALLIC-REC. (AU)

Introduction: since 2002, the Grupo Argentino para el Tratamiento de la Leucemia Aguda (GATLA) has been implementing protocols from the Berlin-Frankfurt-Münster (BFM) group as the standard treatment for relapses of acute lymphoblastic leukemia (ALL). In 2010, BFM developed the IntReALL 10 protocol, implemented in Argentina with the inherent limitations of the region. Population and Methods: we treated a total of 180 patients under 18 years of age between 2010 and 2015 for high-risk relapsed acute lymphoblastic leukemia (ALL) in Argentina following a BFM relapse protocol. This protocol openly compared standard treatment with an innovative (experimental) therapeutic approach that included Clofarabine. Out of these, 171 patients were assessable, with 78 patients being centrally randomized in a clinical trial, and 93 were assigned to one of the arms based on the treating group's criteria (prospective cohort). The cohort where the treatment assignment had not been randomized, was analyzed with adjustments for gender, age, and the presence or absence of Down Syndrome, Philadelphia Chromosome, and T-cell immunophenotype. Results: patients who received the experimental treatment had worse outcomes (double the five-year mortality) compared to those who received the standard treatment. This difference reached statistical significance in the clinical trial (p=0.001) and the prospective cohort (p=0.0009). Conclusions: our results support the continuation of the standard arm in BFM-type protocols for relapsed ALL treatment and were consistent with the conclusions of the ALLIC-REC group. (AU)

Hematological, histopathological and oxidative stress responses to n-hexane extract of Terminalia catappa nuts in leukemia-induced Wistar rats

Background: Terminalia catappa nut possesses antioxidant and anticancer properties, but its effects on leukemia are unclear. This study investigates the effects of n-hexane extract of Terminalia catappa nut (TCN) on some hematological parameters, oxidative stress markers, and bone/spleen histopathology in a Wistar rat model of benzene-induced leukemia. Methods: Leukemia was induced in Wistar rats with 0.2 ml/kg Benzene solution and treated with 200, 400 or 800 mg/kg/day of Terminalia catappa nut extract (TCN) for 42 days and with 5-fluorouracil (20 mg/kg) via intraperitoneal injection twice a week for 6 weeks. Hematological parameters, antioxidant markers, and bone and spleen histology were analysed. Results: All TCN doses significantly lowered elevated WBCs by 32-53% and normalized RBC parameters compared to leukemic controls, mitigating cancer-induced anemia. TCN also exhibited potent antioxidant effects by enhancing SOD, GSH, and catalase while reducing MDA versus untreated rats. Bone marrow analysis revealed TCN conferred dose-dependent benefits on cellularity and architecture, reducing myeloid blasts and leukocyte infiltration. A near-normal bone microarchitecture was attained with the highest TCN dose. Similarly, TCN elicited marked improvements in splenic cytoarchitecture and attenuation of hypercellularity, lymphocytic infiltration and megakaryocytes compared to leukemic controls in a dose-dependent manner. Conclusions: Terminalia catappa nut extract demonstrated anti-leukemic, haematopoietic, antioxidant, and organ protective effects in leukemic Wistar rats induced with benzene solution, supporting its potential as an adjuvant therapeutic agent.

Hospitalization expenses of adult leukemia patients with relapse based on quantile regression / 公共卫生与预防医学

Objective Based on quantile regression analysis, the influencing factors of relapse hospitalization expenses of adult leukemia patients were analyzed. Methods Analyze the composition and influencing factors of hospitalization expenses for leukemia recurrence patients in our hospital. Results From 2017 to 2022 , the per capita hospitalization cost for leukemia patients with recurrence showed an increasing trend year by year. The results of quantile regression model showed that age, payment method , length of stay, times of stay, operation and complications had an impact on the hospitalization expenses of patients at different quantiles, and the difference between different quantiles was statistically significant (P<0.05). Conclusions The quantile regression method can more clearly reflect the distribution of the variables of each factor , we can reduce the hospitalization expenses of patients by improving the coverage rate of medical insurance and controlling the length of stay.

Strategy of Targeted T Cell Immunotherapy for Acute Myeloid Leukemia / 肿瘤防治研究

T cell dysfunction is a common feature in patients with acute myeloid leukemia (AML). The up-regulation of immune checkpoint (IC) proteins resulting in T cell exhaustion is a key reason for T cell dysfunction. Immunotherapy with IC inhibitors exerts a remarkable effect on AML. However, due to the heterogeneity of T cell exhaustion and other factors that impair T cell function in patients with AML, the optimization of targeted T cell immunotherapy strategy for AML might be based on the multidimensional investigation of immune deficiency with different T cell subtypes.

Clinical analysis of lung transplantation for lung chronic graft-versus-host disease after hematopoietic stem cell transplantation / 器官移植

<b>Objective</b> To evaluate clinical efficacy of lung transplantation for lung chronic graft-versus-host disease (cGVHD) after hematopoietic stem cell transplantation (HSCT). <b>Methods</b> Clinical data of 12 patients undergoing lung transplantation for lung cGVHD were retrospectively analyzed. Preoperative clinical manifestations and involved organs of patients were analyzed. The lung function before and after lung transplantation was compared, and the survival of patients after lung transplantation was analyzed. <b>Results</b> Eleven patients underwent HSCT due to primary hematological malignancies, including 9 cases of leukemia, 1 case of myelodysplastic syndrome, 1 case of lymphoma. And 1 case underwent HSCT for systemic lupus erythematosus. Among 12 cGVHD patients, skin involvement was found in 8 cases, oral cavity involvement in 5 cases, gastrointestinal tract involvement in 4 cases and liver involvement in 3 cases. All 12 patients developed severe respiratory failure caused by cGVHD before lung transplantation, including 9 cases of typeⅡ respiratory failure and 3 cases of type Ⅰ respiratory failure. Two patients underwent right lung transplantation, 2 cases of left lung transplantation and 8 cases of bilateral lung transplantation. The interval from HSCT to lung transplantation was 75 (19-187) months. Upon the date of submission, postoperative follow-up time was 18 (7-74) months. Ten patients survived, 1 died from severe hepatitis at postoperative 22 months, and 1 died from gastrointestinal bleeding at postoperative 6 months. No recurrence of primary diseases was reported in surviving patients. <b>Conclusions</b> Lung transplantation is an efficacious treatment for lung cGVHD after HSCT, which may prolong the survival time and improve the quality of life of the recipients.

Progress of gene-mediated precision immunotherapy in the treatment of acute myeloid leukemia / 中国药科大学学报

@#Acute myeloid leukemia (AML) is a disease caused by abnormal cloning of hematopoietic stem cells in the bone marrow, which leads to accumulation of a large number of abnormally differentiated myeloid cells. It is difficult to cure by traditional treatment. The successful application of chimeric antigen receptor T cell (CAR-T) immunotherapy indicates that the treatment of hematological tumors has entered a new stage of precision immunotherapy. However, CAR-T immunotherapy has been found to have many problems in clinical applications, including long treatment cycle, expensive prices, off-target effects, cytokine release syndrome, etc. Therefore, it is necessary to expand the application of CAR or adopt improved measures to enhance the therapeutic effect. This article reviews the new strategies for genetic engineering modification of CAR immune cells and the research progress and application of in situ programming to generate CAR-T, and besides, briefly introduces the new methods about the delivery of gene drugs in vivo, aiming to provide new ideas and theoretical basis for expanding and improving the application of precision immunotherapy in AML.

Meta-analysis on efficacy and safety of thalidomide combined with CAG regimen in the treatment of elderly patients with acute myeloid leukemia / 中国药房

OBJECTIVE To systematically evaluate the efficacy and safety of thalidomide combined with aclacinomycin， granulocyte colony-stimulating factor and cytarabine （CAG） regimen in the treatment of elderly patients with acute myeloid leukemia （AML）. METHODS CNKI， Wanfang data， VIP， Sino Med， PubMed， Embase， the Cochrane Library and Web of Science were searched comprehensively from the inception to Aug. 27th， 2023. Randomized controlled trials （RCTs） about thalidomide combined with CAG regimen （trial group） versus CAG regimen （control group） in the treatment of elderly AML patients were collected， and RevMan 5.3 software was used for meta-analysis of included studies. RESULTS Finally， 7 RCTs were included， with a total of 601 patients， including 307 patients in the trial group and 294 patients in the control group. Meta-analysis results showed that the trial group was superior to the control group in enhancing the overall response rate [Z＝4.75， P＜0.000 01， OR＝2.80， 95%CI （1.83，4.28）]， complete remission rate [Z＝2.82， P＝0.005， OR＝1.61， 95%CI （1.16， 2.25）]， and improving platelet count [Z＝2.70， P＝0.007， MD＝64.02， 95%CI （17.53， 110.51）]， vascular endothelial growth factor [Z＝13.63，P＜0.000 01， MD＝－65.17， 95%CI（－74.54， －55.80）]， vascular endothelial growth factor receptor [Z＝12.03， P＜ 0.000 01， MD＝－499.01， 95%CI （－580.31， －417.71）] and basic fibroblast growth factor [Z＝4.17， P＜0.000 1，MD＝－0.23， 95%CI（－0.35， －0.12）]. And there was no statistical difference between the trial group and the control group in the incidence of adverse drug reaction [Z＝0.99， P＝0.32， OR＝0.52， 95%CI（0.14，1.89）]， nausea and vomiting [Z＝ 1.06， P＝0.29， OR＝0.66， 95%CI （0.30，1.43）]， constipation or diarrhea [Z＝0.92， P＝0.36， OR＝0.65， 95%CI（0.26， 1.63）]， drowsiness [Z＝1.38， P＝0.17， OR＝0.57， 95%CI（0.26， 1.27）] or myelosuppression [Z＝0.88，P＝0.38，OR＝0.68，95%CI（0.28， 1.62）]. CONCLUSIONS The combination of thalidomide and CAG regimen in the treatment of elderly AML patients can significantly improve clinical efficacy and has high safety.