The Epidemiological Particularities of Malignant Hemopathies in French Guiana: 2005-2014.

French Guiana is a French Overseas territory with singular features: it has a high prevalence of HIV and HTLV-1, its population is ethnically mixed, with widespread poverty, and up to 20% of the population lives in geographic isolation. In this context, we used registry data to estimate incidence and mortality due to hematological malignancies and to compare them with France and tropical Latin America. ICD codes C90 and C88 were compiled between 2005 and 2014. The direct standardization of age structure was performed using the world population. Survival analysis was performed, and Kaplan-Meier curves were drawn. The overall standardized incidence rate was 32.9 per 100,000 male years and 24.5 per 100,000 female years. Between 2005 and 2009, the standardized incidence rate was 29.6 per 100,000 among men and 23.6 per 100,000 among women, and between 2010 and 2014, it was 35.6 per 100,000 among men and 25.2 per 100,000 among women. Multiple myeloma/plasmocytoma and mature t/NK cell lymphomas, notably adult t-cell lymphoma/leukemia due to HTLV-1 infection, were the two most common hematologic malignancies and causes of death. Non-Hodgkin's lymphoma incidence estimates were greater than global estimates. After adjusting for age, sex, and type of malignancy, people born in a foreign country independently had a poorer case-fatality rate, presumably reflecting difficulties in accessing care. The epidemiology of hematological malignancies in French Guiana has features that distinguish it from mainland France or from Latin America. The incidence of multiple myeloma and adult t-cell lymphoma/leukemia was significantly greater in French Guiana than in France or other Latin American countries.

Alteraciones de la hemostasia en pacientes graves con hemopatías malignas: trastornos trombóticos / Altered haemostasis in critically ill patients with blood malignancies: t hrombotic disorders

Introducción: La enfermedad trombótica asociada a las hemopatías malignas es subestimada en el paciente grave; en ocasiones queda a la sombra de las hemorragias que presentan estos enfermos. Su diagnóstico y tratamiento constituyen un desafío para lograr el aumento de la sobrevida en las unidades de cuidados críticos Objetivos: Analizar los aspectos generales de las alteraciones trombóticas en pacientes graves con hemopatías malignas. Métodos: Se realizó una investigación bibliográfico-documental acerca del tema. Se consultaron en las bases de datos de SciELO y Pubmed, los artículos publicados en los últimos diez años. Análisis y síntesis de la información: Se describen los aspectos generales vinculados con el mecanismo de la coagulación, sus criterios diagnósticos; así como la evolución del paciente con trombosis que ingresan en las unidades de cuidados intensivos. Conclusiones: El conocimiento de las alteraciones tromboembólicas en los pacientes con hemopatías malignas permite un monitoreo adecuado y la creación de estrategias individuales para mejorar la sobrevida de estos enfermos en la la unidad de cuidados intensivos.

Introduction: Thrombotic disease associated with malignant hemopathies is underestimated in critically ill patients; sometimes it remains in the shadow of the hemorrhages that these patients present. Its diagnosis and treatment constitute a challenge to achieve increased survival in these patients in critical care units Objectives: To analyze the general aspects of thrombotic alterations in seriously ill patients with malignant hemopathies. Methods: A bibliographical-documentary research on the subject was carried out. The SciELO and Pubmed databases of the last ten years were consulted. Analysis and synthesis of the information: General aspects related to the coagulation mechanism, its diagnostic criteria are described; as well as the evolution of the patient with thrombosis who is admitted to the intensive care unit. Conclusions: Knowledge of thromboembolic alterations in patients with malignant hemopathies allows adequate monitoring and the creation of individual strategies to improve the survival of these patients in the ICU.

Monitoreo de las insuficiencias orgánicas en pacientes graves con hemopatías malignas por el uso de las células CAR-T / Monitoring of organ failures in critically ill patients with blood malignancies by the use of CAR - T cell

Introducción: La inmunoterapia con células T modificadas con receptor quimérico antígeno específico es un tratamiento prometedor para hemopatías malignas. Sin embargo, la activación dirigida de la respuesta inmunitaria desata en ciertos casos complicaciones específicas graves y mortales. Objetivos: Describir el monitoreo de las complicaciones por el uso de las células T con receptor antígeno quimérico en pacientes graves con hemopatías malignas. Métodos: Se realizó una investigación bibliográfico documental acerca del tema. Se consultaron las bases de datos de SciELO y PubMed de los últimos cinco años. Conclusiones: Se describieron las complicaciones derivadas de la terapia con células inmunoefectoras, que aumentan el desarrollo de insuficiencias orgánicas, a través del síndrome de liberación de citoquinas y el síndrome de toxicidad neurológica. El tratamiento se basó en establecer medidas de monitorización y soporte, tratamiento con anticonvulsivantes, corticosteroides e ingreso en los servicios de medicina intensiva de forma precoz. Se disminuyó el riesgo en la aparición de complicaciones y muerte con un adecuado monitoreo de las insuficiencias orgánicas derivadas de la inmunoterapia de células T con receptor antígeno quimérico.

Introduction: Immunotherapy with T-cells modified with antigen-specific chimeric receptor is a promising treatment for malignant hemopathies. However, the targeted activation of the immune response in certain cases unleashes specific severe and fatal complications. Objectives: To describe the monitoring of complications from the use of CAR T-cells in critically ill patients with blood malignancies. Methods: A bibliographical-documentary research on the subject was carried out. The SciELO and Pubmed databases of the last five years were consulted. Conclusions: Complications derived from the therapy with immunoeffector cells are described, which increase the development of organ failures, through the cytokine release syndrome and the neurological toxicity syndrome. Treatment is based on monitoring and support measures, treatment with anticonvulsants, corticosteroids, and early admission to intensive care. With adequate monitoring of organ failure derived from chimeric antigen receptor T-cell immunotherapy, a decreased risk of complications and death in these patients was carried out.

Inside the Black Box: A Narrative Review on Comprehensive Geriatric Assessment-Driven Interventions in Older Adults with Cancer.

There is a consensus that the use of comprehensive geriatric assessment (CGA) is good clinical practice for older patients with solid tumors or hematological malignancies. To be complete, a CGA must include a geriatric assessment and an intervention plan. According to the SIOG consensus, a CGA should assess several domains: functional status, comorbidity, cognition, mental health status, fatigue, social status and support, nutrition, and the presence of geriatric syndromes. Progress has been made in the definition of the best way to detect problems, but the benefits are mostly based on prognosis stratification and on the adaptation of cancer treatment. The present review aims to evaluate the level of evidence regarding geriatric interventions proposed following the detection of a problem in cancer patients in each domain mentioned in the SIOG consensus. An online search of the PubMed database was performed using predefined search algorithms specific for each domain of the CGA. Eligible articles had to have well-defined interventions targeting specific domains of the CGA. We screened 1864 articles, but only a few trials on single-domain interventions were found, and often, these studies involved small groups of patients. This review highlights the scarcity of published studies on this topic. The specific impacts of CGA-based interventions have not yet been demonstrated. Multi-domain interventions seem promising, especially when they are based on global assessments. However, standardization seems difficult considering the lack of evidence for each domain. New studies are necessary in multiple care contexts, and innovative designs must be used to balance internal and external validity. An accurate description of the intervention and what "usual care" means will improve the external validity of such studies.

Teenagers and young adults with a past of allogenic hematopoietic stem cell transplantation are at significant risk of chronic kidney disease.

BACKGROUND: Allogenic hematopoietic stem cell transplantation (aHSCT) remains the treatment of choice for some malignant hemopathies in children, albeit with the risk of long-term consequences, including chronic kidney disease (CKD). METHODS: In our single tertiary referral center, we retrospectively assessed the long-term renal outcome in a cohort of children and adolescents who had undergone aHSCT for malignant hemopathies between 2003 and 2017. We distinguished glomerular and tubular dysfunctions and assessed the accuracy of the most common formula(s) to estimate glomerular filtration rate (GFR) during standard clinical follow-up. RESULTS: Among the 166 patients who had received aHSCT, 61 underwent kidney functional assessment 1 to 10 years post-transplantation. Twenty-seven patients (44.3%) had a CKD with glomerular impairment, including 20 patients with a GFR < 90 mL/min/1.73 m2, and among these, 5 patients < 60 mL/min/1.73 m2. Patients with tubular signs had a significantly higher baseline GFR: 112 mL/min/1.73 m2 [100; 120] versus 102 [99.0; 112.5] for patients without kidney involvement, and 76 [61; 86] for patients with CKD (p < 0.01). Schwartz, CKiDU25, and EKFC formulas significantly overestimated mGFR, with a P30% ≤ 30%, which could lead to overlooking CKD diagnosis in this population. No patient reached kidney failure. CONCLUSIONS: In conclusion, our study shows that CKD represents an important long-term sequela for children and adolescents who undergo aHSCT for malignant hemopathies, either with glomerular dysfunction or with the more insidious tubular dysfunction which could potentially impact growth. These patients could benefit from specialized long-term nephrology follow-up. A higher resolution version of the Graphical abstract is available as Supplementary information.

Cancer - Associated Thrombosis - A Study of Cases.

Research shows that the presence of cancer increases the likelihood of developing venous thromboembolism (pulmonary thromboembolism and deep vein thrombosis) from as much as fourfold up to sevenfold. It is imperative that after early diagnosis we treat cancer-associated thrombosis with grave seriousness in order to reduce its morbidity and mortality. We present 14 case reports of patients with cancer-associated thrombosis including thrombosis related to malignant hemopathies.

Identifying frailty in clinically fit patients diagnosed with hematological malignancies using a simple clinico-biological screening tool: The HEMA-4 study.

INTRODUCTION: This study aims to develop and validate a simple score to estimate survival in the older population suffering from malignant hemopathies. METHODS: We prospectively recruited 285 patients, aged ≥65 years, admitted to receive chemotherapy. At inclusion, a geriatric assessment was performed. Cox proportional hazards models were performed to assess correlations between vulnerabilities and one-year survival. We developed a frailty score, HEMA-4, based on the most powerful prognostic factors. It was externally confirmed with an independent cohort. RESULTS: In the development cohort, 206 patients were evaluable. Mean age was 76 years (range 65-90). The HEMA-4 score was created based on four independent predictive factors for survival: cognitive impairment (MMSE<27), comorbidities (≥2 on Charlson comorbidity index), CRP (≥10 mg/L) and low albumin level (<35 g/L). The population was stratified into three groups: good prognosis (score = 0-1, n = 141), intermediate prognosis (score = 2, n = 37) and poor prognosis (score = 3-4, n = 28). Their respective one-year survival was 74%, 51% (HR = 2.30; 95% CI =1.31-4.05; p < 0.01) and 36% (HR = 3.95; 95% CI =2.23-6.98; p < 0.01). In the validation cohort (n = 25), the one-year survival was 78% in the good prognosis group (n = 9) and 50% in the intermediate prognosis group (n = 6). The poor prognosis group had a median survival of four months in the development cohort and six months in the validation cohort (n = 10). CONCLUSION: The HEMA-4 score is a simple score that combines cognitive impairment, comorbidities, inflammation and low albumin level. Our data suggest that it predicts survival among older patients suffering from malignant hemopathies referred to receive chemotherapy regardless of their chronological age.

Caracterización inmunofenotípica de pacientes con leucemia mieloide aguda / Immunophenotypic characterization of patients with acute myeloid leukemia

Introducción: la leucemia mieloide aguda incluye un grupo heterogéneo de neoplasias caracterizadas por una expansión clonal de mieloblastos, cuya clasificación involucra varios criterios, incluidos los inmunológicos. Objetivo: caracterizar el inmunofenotipo de los pacientes con leucemia mieloide aguda evaluados en el Instituto de Hematología e Inmunología. Métodos: se realizó un estudio descriptivo transversal de los pacientes diagnosticados con este tipo de leucemia, cuyas muestras de sangre fueron procesadas en el Departamento de Inmunología en el período 2008-2012. Se usó un ultramicrométodo inmunocitoquímico que utiliza un panel de anticuerpos monoclonales específicos de antígenos mieloides y linfoides. Las variables analizadas fueron: edad, sexo, subtipo de leucemia y expresión de marcadores inmunológicos, cuyas asociaciones fueron analizadas con los estadígrafos Chi-cuadrado y coeficiente de correlación de Spearman. Resultados: se estudiaron 58 pacientes, 28 del sexo femenino y 30 del masculino. El grupo de edad predominante fue de 0 a 9 años con una mediana de 26 años. El subtipo M4 resultó el más frecuente (30,4 por ciento). Los subtipos M4 y M7 predominaron en niños, mientras que el M0, predominó en adultos, con diferencias estadísticamente significativas (p d»0,05). La combinación de los antígenos panmieloides CD13 y CD33 se presentó en el 91 por ciento de los enfermos. Las combinaciones de CD13/CD33, CD14/CD15, CD33/CD14 y CD33/CD15 mostraron correlación significativa. En el 20,6 por ciento de los pacientes evaluados, fueron detectados, además, antígenos linfoides. No se encontraron diferencias significativas en cuanto al sexo y la edad. El antígeno CD7 fue el más expresado, seguido de los antígenos: CD3, CD20, CD22 y CD79, en igual proporción. Conclusiones: el inmunofenotipaje celular demostró ser un procedimiento útil para confirmar el diagnóstico morfológico y clínico de la leucemia mieloide aguda

Introduction: Acute myeloid leukemia (AML) is a heterogeneous group of diseases characterized by clonal expansion of myeloblasts; its classification involves several criteria, including the immunological one. Objective: To characterize the immunophenotype of patients with acute myeloid leukemia who were evaluated at the Institute of Hematology and Immunology. Methods: A descriptive study of patients diagnosed with this kind of leukemia, whose blood samples were processed at the Department of Immunology during 2008-2012. An immunocytochemical ultramicromethod that uses a panel of monoclonal antibodies specific for myeloid and lymphoid antigens was applied. The variables analyzed were age, sex, subtype of leukemia and expression of immunological markers; their association was analyzed with the Chi-square test and Spearman's rank correlation coefficient. Results: The study covered 58 patients; 28 were males and 30 females. The predominant age group was 0 - 9 years with a median of 26 years. M4 subtype was the most common (30,4 percent). M4 and M7 subtypes predominated in children, while M0 predominated in adults with statistically significant differences (p d» 0,05). The combination of pan-myeloid antigens CD13 and CD33 was present in 91 percent of patients. Combinations of CD13/CD33, CD14/CD15, CD33/CD14 and CD33/CD15 showed significant correlation. In 20,6 percent of patients tested, lymphoid antigens were also detected. In this group, no significant differences by gender and age. CD7 antigen was the most expressed followed by antigens: CD3, CD20, CD22 and CD79, in equal proportion. Conclusions: Immunophenotyping of leukemia cells appeared as a useful tool to confirm the morphological and clinical diagnosis of acute myeloid leukemia

Frecuencia de hepatitis B y C en niños con hemopatías malignas / Frequency of hepatitis B and C with malignant hemopathies

Se estudiaron 101 niños con hemopatías malignas diagnosticados y atendidos en el Instituto de Hematología e Inmunología (IHI). Se investigó la frecuencia del antígeno de superficie de la hepatitis B (AgsHB) y la presencia de anticuerpos contra el virus de la hepatitis C (AcVHC). En la relación del AgsHB con el diagnóstico, se evaluó el tiempo de evolución de la enfermedad, la etapa de tratamiento y la transfusión de componentes sanguíneos. A todos los pacientes se les determinó el AgsHB y el AcVHC con los estuches UMELISA. De los pacientes estudiados, 7 (6,93 %) fueron positivos para el AgsHB y 2 (1,98 %) fueron positivos al AcVHC, para un total de 9 pacientes (8,91 %). Debido a que solo 2 pacientes fueron positivos al AcVHC, no se realizó el análisis estadístico de estos parámetros.

110 children with malignant hemopathies that were diagnosed and seen at the Institute of Hematology and Immunology were studied. The frequency of hepatitis B surface antigen (HBsAg) and the presence of antibodies against the hepatitis C virus (HCVAbs) was investigated. In the relation of HBsAg to the diagnosis, the time of evolution of the disease, the treatment stage and the transfusion of blood components were evaluated. All the patients were determined HbsAg and HCVAb with UMELISA kits. Of the studied patients, 7 (6.93 %) were positive for HbsAg and 2 (1.98 %) tested positive for HCVAb, for a total of 9 patients (8.91 %). As only 2 patients were positive for VHCAb, the statistical analysis of these parameters was not made.