Microorganismos aislados de hemocultivos en niños con leucemia y linforma: Seis años de seguimiento / Microorganisms isolated from blood cultures in children with leukemia and lymphoma: six years of follow-up

Las Leucemias y linfomas constituyen las enfermedades oncológicas más frecuentes en pediatría y las bacteriemias representan infecciones graves en estos pacientes. Objetivos: describir los microorganismos aislados de sangre en pacientes con leucemia aguda o linfoma pediátrico; comparar la incidencia de aislamientos según enfermedad de base; detallar las variaciones en la incidencia de dichos aislamientos y la evolución de su resistencia antimicrobiana. Estudio retrospectivo, observacional. Se incluyeron 823 episodios de bacteriemia en 467 pacientes pediátricos, entre julio-2016 y junio-2022, dividido en tres períodos (período-1: años 2016- 2018, período-2: años 2018-2020, período-3: años 2020-2022). Se aislaron 880 microorganismos: 55,3% gram negativos (GN), 40% gram positivos (GP) y 4,7% levaduras. En GN predominaron: enterobacterias (72%) y en GP: estreptococos del grupo viridans (SGV) (34,1%). Se encontró asociación entre LLA-enterobacterias (p=0,009) y LMA-SGV (p<0,001). Hubo aumento de GN entre los períodos 1 y 3 (p=0,02) y 2 y 3 (p=0,002) y disminución de GP entre 2 y 3 (p=0,01). Se registraron los siguientes mecanismos de resistencia: BLEE (16,4%), carbapenemasas: KPC (2,5%); MBL (2,7%) y OXA (0,2%); meticilinorresistencia en Staphylococcus aureus (20%) y estafilococos coagulasa negativos (95%), vancomicina resistencia en Enterococcus spp. (39%), SGV no sensibles a penicilina (44%) y a cefotaxima (13%). Hubo aumento de MBL entre los períodos 1 y 2 (p=0,02) y una tendencia en disminución de sensibilidad a penicilina en SGV entre el 1 y 3 (p=0,058). El conocimiento dinámico y análisis de estos datos es esencial para generar estadísticas a nivel local, fundamentales para el diseño de guías de tratamientos empíricos (AU)

Leukemias and lymphomas are the most common cancers in children and bacteremia is a severe infection in these patients. Objectives: to describe the microorganisms isolated from blood in pediatric patients with acute leukemia or lymphoma; to compare the incidence of isolates according to the underlying disease; and to detail the variations in the incidence of these isolates and the evolution of their antimicrobial resistance. Retrospective, observational study. We included 823 episodes of bacteremia in 467 pediatric patients seen between July-2016 and June-2022, divided into three periods (period-1: 2016- 2018, period-2: 2018-2020, period-3: 2020-2022). A total of 880 microorganisms were isolated: 55.3% were gram-negative (GN), 40% gram-positive (GP) and 4.7% yeasts. In GN there was a predominance of: enterobacteria (72%) and in GP viridans group streptococci (VGS) (34.1%). An association was found between ALL-enterobacteria (p=0.009) and AML-VGS (p<0.001). There was an increase in GN between periods 1 and 3 (p=0.02) and 2 and 3 (p=0.002) and a decrease in GP between 2 and 3 (p=0.01). The following resistance mechanisms were recorded: BLEE (16.4%), carbapenemases: KPC (2.5%), MBL (2.7%), and OXA (0.2%); methicillin resistance in Staphylococcus aureus (20%) and coagulase negative staphylococci (95%), vancomycin resistance in Enterococcus spp. (39%), VGS resistant to penicillin (44%) and to cefotaxime (13%). There was an increase in MBL between periods 1 and 2 (p=0.02) and a decreasing trend in penicillin sensitivity in VGS between periods 1 and 3 (p=0.058). Dynamic knowledge and analysis of these data is essential to generate statistics at the local level, which is fundamental for the design of empirical treatment guidelines (AU)

Effect and safety of 10-day decitabine-containing conditioning regimen for allogeneic hematopoietic stem cell transplantation in 31 patients with acute myeloid leukemia/myelodysplastic syndrome / 中华血液学杂志

Objective: To investigate the early effect and safety of allogeneic hematopoietic stem cell transplantation (allo-HSCT) with a 10-day decitabine-containing conditioning regimen in the treatment of acute myeloid leukemia (AML) /myelodysplastic syndrome (MDS) . Methods: From April 2021 to May 2022, 31 AML/MDS patients who received allo-HSCT with a 10-day decitabine-containing conditioning regimen were analyzed. Results: AML (n=10), MDS-AML (n=6), CMML-AML (n=1), and MDS (n=14) were identified in 31 patients, 16 males, and 15 females, with a median age of 41 (20-55) yr. Neutrophils and platelets were successfully implanted in 31 patients (100%), with a median implantation duration of 12 (9-30) and 14 (9-42) days, respectively. During the preconditioning period, 16 patients (51.6%) developed oral mucositis, with 15 cases of Ⅰ/Ⅱ grade (48.4%) and one case of Ⅲ grade (3.2%). After transplantation, 13 patients (41.9%) developed CMV viremia, six patients (19.4%) developed hemorrhagic cystitis, and four patients (12.9%) developed a local infection. The median time of acute graft versus host disease (aGVHD) following transplantation was 33 (12-111) days. The cumulative incidence of aGVHD and Ⅲ/Ⅳ grade aGVHD was 41.9% (95% CI 26.9%-61.0%) and 22.9% (95% CI 13.5%-47.5%), respectively. There was no severe cGVHD, and mild and moderate chronic GVHD (cGVHD) incidence was 23.5% (95% CI 12.1%-43.6%). As of November 30, 2022, only one of the 31 patients had relapsed, with a 1-yr cumulative relapse rate (CIR) of 3.2% (95% CI 0.5%-20.7%). There was only one relapse patient death and no non-relapse deaths. The 1-yr overall survival (OS) and disease-free survival (DFS) rates were 92.9% (95% CI 80.3%-100%) and 96.8% (95% CI 90.8%-100%), respectively. Conclusions: A 10-day decitabine-containing conditioning regimen for allo-HSCT reduced relapse and was safe and feasible in treating AML/MDS.

The Relationship between Occurrence of aGVHD in Patients with Acute Myeloid Leukemia after Allogeneic Hematopoietic Stem Cell Transplantation and Immune Cell Components in Graft / 中国实验血液学杂志

OBJECTIVE@#To explore the relationship between occurrence of acute graft-versus-host disease (aGVHD) and various immune cell composition in patients with acute myeloid leukemia (AML) after allogeneic hematopoietic stem cell transplantation (allo-HSCT).@*METHODS@#The clinical data of 104 patients with AML undergoing allo-HSCT in our hospital were retrospectively analyzed, and the hematopoietic reconstitution and occurrence of GVHD were analyzed. Flow cytometry was used to detect the proportion of various types of immune cells in the grafts, the number of graft composition in patients with different degrees of aGVHD was calculated and compared, and to analyze the correlation between the severity of aGVHD in AML patients after allo-HSCT and the immune cell components in the graft.@*RESULTS@#There was no significant difference in the time of hematopoietic reconstitution between the high number group of total number of nucleated cells (TNC) and the low number group, while the time of neutrophil and platelet reconstruction in the high number of CD34 group was significantly faster than that in the low number of CD34 group (P＜0.05), and the total hospital stay also tends to be shorten. Compared with patients in 0-Ι aGVHD group, both HLA-matched and HLA-haploidentical transplantation, the infusion amounts of CD3+ cells, CD3+CD4+ cells, CD3+CD8+ cells, NK cells and CD14+ monocytes were higher in patients of Ⅱ-Ⅳ aGVHD group， but the difference was not statistically significant (P>0.05); In addition, in patients with HLA-haploidentical transplantation, the number of CD4+CD25+ cells in Ⅱ-Ⅳ aGVHD group was significantly lower than that in 0-Ι aGVHD group (P<0.05), and the same trend was also observed in HLA-matched transplanted patients, but the difference was not significant (P=0.078).@*CONCLUSION@#High number of CD34+ cells in the graft is beneficial to hematopoietic reconstitution in AML patients. To a certain degree, high number of CD3+ cells, CD3+CD4+ cells, CD3+CD8+ cells, NK cells and CD14+ cells tend to increase the occurrence of aGVHD, but high number of CD4+CD25+ regulatory T cells is beneficial to reduce the incidence of aGVHD in AML patients.

Trastornos mieloproliferativos y leucemia en síndrome de Down. Presentación de dos casos clínicos en el período neonatal / Myeloproliferative disorders and leukemia related to Down syndrome. Report of two clinical cases in neonatal period

ElsíndromedeDownpredisponeatrastornosmieloproliferativos. Se estima que del 5 % al 30 % de los neonatos con esta condición desarrollarán mielopoyesis anormal transitoria. El tratamiento no está estandarizado; la exanguinotransfusión y la citarabina podrían ser efectivos. Se describen dos casos de pacientes con síndrome de Down, quienes durante el período neonatal presentaron leucemia mieloide aguda y mielopoyesis anormal transitoria, los tratamientos utilizados y sus desenlaces. Se considera que la sospecha y el diagnóstico temprano de esta entidad son factores determinantes en el pronóstico.

Down syndrome predisposes to haematological disorders. It is estimated that 5-30% of neonates with this condition will develop transient abnormal myelopoiesis. Treatment is not standardized; exchange transfusion and the use of cytarabine could be effective. We present two clinical cases of patients with Down syndrome, who during the neonatal period showed acute myeloid leukemia and transient abnormal myelopoiesis, the treatments used and their outcomes. Suspicion and early diagnosis of this entity are considered determining factors in prognosis.

Clinical Characteristics and Prognosis of Patients with Hematological Malignancies Superimposed with Solid Tumors / 中国实验血液学杂志

OBJECTIVE@#To investigate the clinical characteristics and prognosis of hematological malignancies superimposed patients with solid tumors.@*METHODS@#The clinical data of 30 patients with more than two kinds of malignancy (the second is hematological malignancy) from October 2011 to October 2020 in Department of Hematology, Jiangning Hospital Affiliated to Nanjing Medical University were collected and analyzed retrospectively. The overall survival time was used as the prognostic evaluation standard, and the survival of patients were analyzed by KaplanMeier method. Logrank test and Cox regression model were used to carry out univariate and multivariate retrospective analysis on clinical and laboratory parameters of 30 patients.@*RESULTS@#Among 30 cases, 20 were male, 10 were female, the median age of onset of the second tumor was 70 years old. The common types of the secondary hematological malignancies to solid tumors are myelodysplastic syndrome, acute myeloid leukemia, multiple myeloma. Univariate analysis showed that patients' gender, age, type of solid tumors, the onset of interval between two kinds of tumor, chromosome karyotype were not related to do with the patients' overall survival time. Type of hematologic disease, ECOG score were associated with patients' overall survival time, and the multivariate analysis showed that the type of hematologic disease and ECOG score were independent risk factors for patients with poor prognosis.@*CONCLUSION@#Patients superimposed with solid tumors complicated with myelodysplastic syndrome or acute leukemia and ECOG score ≥3 have poor prognosis and shorter overall survival time, which are independent risk factors influencing the prognosis. Bone marrow injury, immune dysfunction and genetic susceptibility after chemoradiotherapy may be the main causes of these diseases.

Urgencias hematooncológicas: desde la perspectiva del intensivista / Hematology/oncology urgencies: from the perspective of the intensivist

Entre los años 2000 y 2016 en Argentina, se reportaron al Registro Oncopediátrico Hospitalario Argentino (ROHA) 22.450 casos de cáncer en niños menores de 15 años de edad. Las Leucemias constituyen la enfermedad oncológica más frecuente, seguida de los Tumores de Sistema Nervioso Central y los Linfomas. Esta distribución es similar a la descripta en los países desarrollados de Europa y Norteamérica. Su tasa de curación a nivel mundial, llega al 80% debido al uso de quimioterapia intensiva, situación que mejora la supervivencia pero que también aumenta la frecuencia de complicaciones. Estas complicaciones pueden ser debidas tanto al propio cáncer como al tratamiento y en ocasiones ser la primera manifestación de la enfermedad oncológica. Los eventos que amenazan la vida en pacientes inmunocomprometidos son mayores que en la población general, y cuando ocurren tienen una mortalidad elevada. El reconocimiento temprano es clave para el resultado en términos de sobrevida y disminución de la mortalidad. Las acciones deberán centrarse al reconocimiento temprano de eventos críticos en pacientes oncológicos. Los pacientes Hemato-Oncológicos constituyen un gran número de ingresos no planificados a las unidades de cuidados intensivos. Uno de cada 4 pacientes requerirá durante su evolución ingreso a Unidades de Cuidados Intensivos. El propósito de este artículo es describir tres de las urgencias oncológicas que requieren con mayor frecuencia admisión en UCI: la presentación y manejo del shock séptico, Shock Cardiogénico y las complicaciones neurológicas en los pacientes con leucemias agudas (AU)

Between 2000 and 2016, 22,450 cases of cancer in children younger than 15 years of age were reported to the Argentine Hospital Registry of Childhood Cancer (ROHA). Leukemia was the most common cancer reported, followed by central nervous system tumors and lymphoma. This distribution is similar to that described in the developed countries of Europe and North America. The worldwide cure rate is up to 80% due to the use of intensive chemotherapy, which improves survival but also increases the complication rate. These complications may be due both to the cancer itself and to the treatment and are sometimes the first manifestation of the disease. Life-threatening events are more common in immunocompromised patients than in the general population, and when they occur, the mortality rate is high. Early recognition is essential for the outcome in terms of survival and decreased mortality. Interventions should focus on early recognition of critical events in cancer patients. Patients with hematology-oncology diseases account for a large number of unplanned admissions to intensive care units (ICU), while one in four of these patients will require admission to the ICU in the course of their disease. The aim of this study was to describe three oncology emergencies that most frequently require ICU admission: septic shock and its management, cardiogenic shock, and neurological complications in patients with acute leukemia (AU)

Leucemia mieloide aguda y policondritis recurrente ¿casualidad o relación causal? / Acute myeloid leukemya and relapsing polychondritis. Chance or causal relationship?

La policondritis recurrente o recidivante es una enfermedad sistémica crónica autoinmune, caracterizada por la inflamación de tejidos cartilaginosos asociada en pocos casos a enfermedades malignas hematológicas. Presentamos el caso de una paciente femenina de 26 años que cursaba concomitantemente con leucemia mieloide aguda (LMA). La manifestación inicial fue una afección cutánea en forma de eritema nodoso, y posteriormente se diagnosticó LMA; durante la fase de aplasia posquimioterapia desarrolló inflamación bilateral del cartílago auricular (condritis auricular) y síndrome vertiginoso con evolución clínica satisfactoria al tratamiento inmunosupresor con glucocorticoides. Conclusiones: Es difícil definir si existe asociación entre la policondritis recidivante y la leucemia mieloide aguda, la quimioterapia o la sumatoria de las dos noxas. Una vez que se establece el diagnóstico se debe iniciar oportunamente la administración de glucocorticoide a altas dosis, ya que pudieran aparecer complicaciones como la necrosis del cartílago y la pérdida de la región afectada. En contraste, el uso de los glucocorticoides tiene una excelente respuesta con modulación completa de la enfermedad, tal como se muestra en el caso presentado(AU)

Relapsing polychondritis is a systemic, chronic and autoimmune disease characterized by the inflammation of cartilaginous tissues. This disease is associated in a few cases with malignant hematological diseases. We present a case of a patient with relapsing polychondritis and concomitantly with acute myeloid leukemia. A 26-year-old female patient, with cutaneous affection as initial manifestation categorized as erythema nodosum. Then she was diagnosed with acute myeloid leukemia. In the aplasia post-chemotherapy phase, the patient developed bilateral inflammation of the ear cartilage (auricular chondritis) and a vertiginous syndrome with satisfactory clinical evolution to immunosuppressive treatment with glucocorticoids. Conclusion: Relapsing polychondritis usually presents with cartilaginous involvement, such as bilateral atrial chondritis, as shown in the case. Early diagnosis and timely treatment are necessary to achieve a good clinical response. Subsequent studies are necessary to evaluate the association between relapsing polychondritis and hematological alterations such as acute myeloid leukemia and the use of chemotherapy(AU)

Leucemia mieloide aguda y pre-eclampsia coexistente: algunas dificultades diagnósticas: a propósito de un caso / Acute myeloid leukemia and coexisting preeclampsia: some diagnosis difficulties: a case report

OBJETIVO: Remarcar la importancia de hacer un adecuado diagnóstico diferencial de la anemia y trombocitopenia en la gestante, ya que en ocasiones enmascaran cuadros tan graves como la leucemia. Presentar un caso de leucemia mieloide aguda con una preeclampsia sobreañadida y describir el proceso diagnóstico y terapéutico llevado a cabo. CASO CLÍNICO: Secundigesta, 25 años, gestante de 37 semanas, con antecedentes de preeclampsia, derivada desde atención primaria por alteración analítica y malestar general. A su llegada a urgencias el cuadro clínico es compatible con un Síndrome de HELLP. Tras el estudio del mismo se llega a la certeza de que se trata de una preeclampsia asociada a una leucemia mieloide aguda que ha simulado los parámetros analíticos de un Síndrome de HELLP. CONCLUSIONES: Es importante el adecuado estudio etiológico de la anemia y trombocitopenia en la gestación. La leucemia exige al clínico un abordaje precoz y multidisciplinar tanto diagnóstico como terapéutico.

OBJECTIVE: To emphasize on the importance of performing a precise differential diagnosis of anaemia and thrombocytopenia during pregnancy, as they can be due to important diseases as leukemia. A case of acute myeloid leukemia associated with preeclampsia is reported, describing the complexity of the diagnostic and therapeutic process. CLINICAL CASE: 25-year-old woman, gravida 2, para 1 (preeclampsia), at 36 weeks of gestation was referred to the emergency department by her primary care physician due to severe disturbance on the blood tests and general discomfort. Initially, a HELLP syndrome was suspected. However, after going in depth in the case, the final diagnosis was preeclampsia associated to acute myeloid leukemia, simulating blood parameters in HELLP syndrome. CONCLUSIONS: It is essential to study deeply and carry out a complete differential diagnosis process of anaemia and thrombocytopenia during pregnancy. Leukemia requires an early multidisciplinary management both for diagnosis and treatment.

Epidemiología y patrones de sensibilidad a los antimicrobianos de microorganismos aislados de hemocultivos en niños con leucemia aguda / Epidemiology and sensitivity patterns of antimicrobials in microorganisms isolated in blood cultures of children with acute leukemia

Las infecciones bacterianas son una de las principales causas de morbimortalidad en los niños con cáncer. Nuestro objetivo fue describir y comparar las características clínicas y los microorganismos causantes de bacteriemias con su sensibilidad antimicrobiana en niños con diagnóstico de LLA y LMA. Se realizó un estudio observacional, descriptivo entre julio-2016 y junio-2018. Se incluyeron todos los episodios de bacteriemia (EpB) en pacientes de 0 a 18 años con diagnóstico de LLA y LMA. Se documentaron datos epidemiológicos y demográficos de los pacientes y datos microbiológicos de los aislamientos de hemocultivos positivos. Se utilizó stata13. Se incluyeron 258 EpB en 167 pacientes; el 55% eran varones. La mediana de edad fue 81 meses (RIC 39-130). En 215 EpB (83%) se registró la presencia de algún tipo de catéter; neutropenia en 193 EpB (75%), neutropenia severa en 98/258 EpB (38%). Se pudo determinar el foco clínico en 152 EpB (59%). Ciento diez pacientes tenían LLA y 57 LMA. En LLA predominaron las enterobacterias, en LMA los cocos gram positivos. Se observó asociación entre LMA y estreptococos del grupo Viridans (p<0,01) y entre LLA y P.aeruginosa (p 0,01). Con respecto a la sensibilidad hubo 11% y 17% de bacilos negativos multirresistentes en LLA y LMA respectivamente. Todos los estafilococos coagulasa negativos fueron meticilino resistentes. La mayoría de los pacientes tenía algún tipo de catéter y neutropenia. Se observó un predominio de enterobacterias con bajos niveles de resistencia antibiótica. Estos resultados son importantes para conocer la epidemiología local y establecer tratamientos empíricos adecuados (AU)

Bacterial infections are one of the main causes of morbidity and mortality in children with cancer. Our aim was to describe and compare the clinical features and bacteremia-causing microorganisms together with their antimicrobial sensitivity in acute lymphocytic (ALL) and acute myelocytic leukemia (AML). A descriptive observational study was conducted between July 2016 and June 2018. All episodes of bacteremia (EpB) in patients between 0 and 18 years of age with ALL and AML were included. All epidemiological and demographic data of the patients and microbiological information of the isolates of the positive blood cultures were recorded. For statistical analysis stata13 was used. Overall 258 EpB in 167 patients were included; 55% were boys. Median age was 81 months (IQR 39-130). In 215 EpB (83%) some type of catheter was involved; neutropenia was observed in 193 EpB (75%) and severe neutropenia in 98/258 EpB (38%). A clinical focus could be determined in 152 EpB (59%). Of all patients, 110 had ALL and 57 AML. The predominant micro-organisms were enterobacteria in ALL and gram-positive cocci in AML. An association was observed between AML and the viridans group of streptococci (p<0.01) and between ALL and P. aeruginosa (p 0.01). Regarding sensitivity, there were 11% and 17% of multiresistant negative bacilli in ALL and AML, respectively. All coagulase-negative staphylococci weer methicillin resistant. The majority of patients had some type of catheter and neutropenia. Predominance of enterobacteria with low levels of resistance to antibiotics was observed. These results are important to understand the local epidemiology and establish adequate empirical therapies (AU)

Infección rino-sinusal por Exserohilum rostratum en un niño con leucemia mieloide aguda / Sinus infection caused by Exserohilum rostratum in a pediatric patient with acute myeloid leukemia

Resumen Los episodios de neutropenia febril son cuadros graves, que requieren un estudio etiológico exhaustivo y un inicio rápido de antimicrobianos. Dentro de los posibles microorganismos causales se encuentran los de origen fúngico, los que, dependiendo de su grado de invasión tisular, pueden llegar a presentar una alta mortalidad. Presentamos el caso de un niño con una leucemia mieloide aguda, que tras su quimioterapia de inducción, presentó un episodio de neutropenia febril, con una infección rino-sinusal por Exserohilum rostratum, hongo filamentoso que raramente se asocia a cuadros patológicos. Se inició rápidamente terapia antifúngica, lo cual, asociado a un aseo quirúrgico precoz, lograron una respuesta clínica favorable, sin complicaciones. Luego del seguimiento, y tras recibir profilaxis secundaria durante los episodios de neutropenia, no ha vuelto a presentar nuevas lesiones ni compromiso rino-sinusal.

Abstract The episodes of febrile neutropenia are severe cases that require an exhaustive etiological study and a quick start of antimicrobial agents. Within the possible microorganisms, fungal origins are also found, and depending on its tissue invasion, they can reach a high mortality rate. A case of a pediatric patient who suffered from acute myeloid leukemia is reported, and after his induction chemotherapy, the patient showed an episode of febrile neutropenia, which matches a rhinosinusal infection caused by Exserohilum rostratum, a filamentous fungi that is uncommonly associated with pathological cases. An antifungal therapy and an early surgical treatment were started, which lead to a positive response, without complications to the patient. After the monitoring and receiving secondary prophylaxis during the episodes of neutropenia, the patient hasn't presented new injuries nor rhinosinusal damage.

Analysis of genetic and clinical characteristics of nine cases of myelodysplastic syndrome with near tetraploid/tetraploidy karyotype / 中华医学遗传学杂志

OBJECTIVE@#To explore the genetic and clinical characteristics of near-tetraploidy/tetraploidy karyotype (NT/T) in patients with myelodysplastic syndrome (MDS).@*METHODS@#Cytogenetic findings of 1576 inpatients with primary MDS were retrospective analyzed, among which 9 were diagnosed with NT/T. Clinical data including gender, age, morphology, genetic feature and prognosis were analyzed.@*RESULTS@#The prevalence of MDS patients with NT/T (NT/T-MDS) among all cases was 0.57%. Karyotyping analysis suggested that eight MDS patients had sole NT/T, while the remainder one had a complex karyotype. In addition to the typical morphology of MDS, NT/T-MDS had unique morphology including huge blast, double-nuclear cell and irregular nuclear membrane. One NT/T-MDS patient gave up therapy, and the remaining eight underwent the first course of treatment, albeit with poor prognosis. Only one patient had complete remission, one had partial remission, three had no remission; and three had converted to acute myeloid leukemia.@*CONCLUSION@#NT/T-MDS is rare and has unique morphology. Generally, NT/T-MDS patients have poor prognosis. However, NT/T cannot be simply classified as high-risk group, but with consideration whether they have affected particular chromosomal structures as well as other clinical data.

Sarcoma mieloide uterino como manifestacion de leucemia aguda mieloide / Uterine myeloid sarcoma as a manifestation of acute myeloid leukemia

RESUMEN Introducción y objetivos: El sarcoma mieloide puede ser la primera manifestación de la leucemia mieloide aguda (LMA), presentarse simultáneamente o constituir una forma de recaída. Material y métodos: Presentamos el caso de una paciente con sarcoma mieloide uterino, como forma de recaída de LMA. Resultados: El diagnóstico se basa en los hallazgos histopatológicos, la inmunohistoquímica y el inmunofenotipo. El tratamiento y el pronóstico son similares a LMA. Conclusión: La afectación uterina por leucemia mieloide extramedular es rara pero debe tenerse en cuenta en el diagnostico diferencial de una masa uterina en aquellas pacientes con antecedentes de LMA.

ABSTRACT Introduction and objectives: Myeloid Sarcoma can present as a first MLA sign, concurrently with or at relapse form. Materials and methods: We present the case of a patient with uterine myeloid sarcoma, as a form of relapse of MLA. Results: The diagnostic is based on the histopathology findings along with immunohistochemistry and immunophenotyping. Conclusion: Uterine involvement due to extramedullary myeloid leukemia is rare but it should be taken into account in the differential diagnosis of a uterine mass in those patients with a history of MLA.

Eficacia del tinidazol en la profilaxis terapéutica de la colitis amebiana en pacientes con leucemia aguda de novo que reciben quimioterapia intensiva / Efficacy of tinidazole in the therapeutic prophylaxis of amoebic colitis in patients with de novo acute leukemia receiving intensive chemotherapy

Resumen Introducción: En México la seroprevalencia de la Entamoeba histolytica es del 8.4%. La amebiasis intestinal en pacientes con leucemia aguda de novo posterior al inicio de quimioterapia (QT), en el Servicio de Hematología del CMN 20 de Noviembre, es del 12%, aún si muestran test coprológico negativo basal. Objetivo: Averiguar si la administración de tinidazol, en pacientes con leucemia aguda y coprológico negativo, al principio de la QT, disminuye la incidencia de colitis amebiana durante la inducción a la remisión. Método: Prospectivo y no comparativo. Enfermos con diagnóstico de leucemia aguda de novo que inician QT de inducción y coprológico inicial. Se indicó tinidazol, 2 g/día durante 5 días en la primera semana de comenzada QT. Se vigilaron hasta que la inducción concluyó y se inició la recuperación hematopoyética. Resultados: 38 pacientes, 15 mujeres y 23 hombres con edad media de 44 años (16-72). Con leucemia aguda linfoblástica 19, con mieloblástica 16 y con promielocítica 3. Casos sin y con amebiasis intestinal, 35 y 3, respectivamente. Los pacientes con amebiasis solo recibieron tinidazol durante 3 días y se dio después de 2 días de empezada la QT. Conclusión: El tinidazol, en pacientes con leucemia aguda de novo que inician QT de inducción, es efectivo en la prevención de la amebiasis intestinal, durante la etapa de inducción, si se administra a 2 g/día, durante cinco días, a partir del día 1 de la QT.

Abstract Introduction: In Mexico, seroprevalence of Entamoeba histolytica is 8.4%. The intestinal amebiasis in patients with acute leukemia of novo, after the start of chemotherapy (CT) in the Hematology Service of the CMN 20 de Noviembre is 12%, even if patients show a negative baseline coprological test. Objective: To find out if the administration of tinidazole, in patients with acute leukemia and negative coprological test, at the beginning of the CT, decreases the incidence of amoebic colitis during the induction to remission. Method: Prospective and not comparative study. Patients with de novo diagnosis of acute leukemia who initiate induction and initial coprological CT. Tinidazole was indicated, 2 g/day for 5 days in the first week of CT started. They were monitored until the induction was concluded and hematopoietic recovery started. Results: 38 patients, 15 women and 23 men with a mean age of 44 years (16-72), with acute lymphoblastic leukemia 19, myeloblastic 16 and promyelocytic 3. Cases without and with intestinal amebiasis were 35 and 3, respectively. Patients with amebiasis only received tinidazole for 3 days and it was given 2 days after the CT started. Conclusion: Tinidazole, in patients with acute de novo leukemia who initiate induction CT, is effective in the prevention of intestinal amebiasis, during the induction stage, if administered at 2 g/day, for five days, starting on day 1 of the CT.

Infección pulmonar por Lophomonas sp en una paciente con leucemia mieloide aguda / Lung infection by lophomonas spp. in a female patient with acute myeloid leukemia

RESUMEN Las infecciones por protozoos son prevalentes a nivel mundial, en particular en pacientes inmunosuprimidos. Comunicamos el caso de una paciente procedente de la ciudad de Viña del Mar, Chile, portadora de leucemia mieloide aguda en quiense confirmó una infección por Lophomonas sp. en lavado bronquioalveolar. Se manejó con antibióticos, pero falleció decomplicaciones de su enfermedad de base. Existe poca literatura disponible respecto a este microorganismo. Concluimos que debe considerarse a Lophomonas sp. como posibilidad diagnóstica si se encuentran protozoos en lavados bronquioalveolares de pacientes inmunosuprimidos.

ABSTRACT Protozoic infections are prevalent worldwide, particularly in immunosuppressed patients. We reported the case of a patient from the city of Viña del Mar, Chile, a carrier of acute myeloid leukemia in whom an infection by Lophomonas sp. was confirmed by bronchoalveolar lavage. She was treated with antibiotics but died of complications of the underlying disease. There is little literature available on this microorganism. We conclude that Lophomonas sp. should be considered as a diagnostic possibility if protozoa are found in bronchoalveolar lavage of immunosuppressed patients.

Epidemiología y factores de riesgo de internación prolongada en niños con leucemia y bacteriemia: estudio de cohorte / Epidemiology and risk factors for prolonged hospital length of stay in children with leukemia and bacteremia: cohort study

Resumen Introducción: La bacteriemia constituye una complicacion frecuente en los niños con cáncer, que se asocia a mayor gravedad, internación prolongada y mortalidad. La internación prolongada condiciona mayor morbilidad y riesgo de adquisición de infecciones intranosocomiales. Objetivo: Analizar factores de riesgo de internación prolongada en niños con leucemia y bacteriemia. Pacientes y Métodos: Cohorte retrospectiva. Se incluyeron niños con leucemia internados en el Hospital Garrahan entre 1/1/2015 y 31/12/2016 con bacteriemia. Se compararon características de pacientes con internaciones menores o mayores a 14 días. Se realizó un análisis bivariado y modelo de regresión logística. Se utilizó Stata 13. Resultados: n = 121. Mediana de edad 59 meses. Tenían leucemia linfoblastica 81 pacientes (67%) y leucemia mieloblástica 40 (33%). Tenían catéter venoso central (CVC) 96 de los niños (79%), neutropenia 94 (78%), neutropenia menor a 100 neutrófilos 79 (65%). La identificación en hemocultivos fue: 55 casos (45%) enterobacterias, 28 (23%) Staphylococcus coagulasa negativa, Streptococcus spp grupo viridans 19 (16%), Pseudomonas aeruginosa 8 (7%). Huo co-infección viral en 14 pacientes (12%).Tuvieron menos de 14 días de internación 71 pacientes (59%) y mayor período 50 (41%). En el análisis multivariado la bacteriemia asociada a CVC (OR 21,73; IC95% 1,2-43,20; p 0,04), neutropenia profunda al ingreso (OR 1,75; IC95% 1,82-1,28; p 0,03) y co-infección viral (OR 27,42; IC95% 2,88-260,83; p 0,004) fueron factores de riesgo de internación > 14 días. Conclusiones: La bacteriemia asociada a CVC, la neutropenia profunda al ingreso y la co-infección se asociaron con una internación igual o mayor a 14 días.

ABSTRACT Introduction: Bacteremia is a frequent complication in children with cancer, which is associated with greater severity, prolonged hospitalization and mortality. Prolonged hospitalization conditions greater morbidity and risk of acquisition of intranosocomial infections. Aim: To describe risk factors for prolonged hospital length of stay in children with leukemia and bacteremia. Methods: Cohort study. Episodes of bacteremia in patients with leukemia at Garrahan Hospital from 1/1/2015 to 31/12/2016 were reviewed. We compared data from patients with a LOS of 14 days or more with those admitted for less than 14 days. Bivariate and logistic regression analysis was performed. We used Stata 13 statistical package. Results: n = 121. Median age 59 months.81 patients (67%) had a diagnosis of acute lymphoblastic leukemia, followed by acute myeloid leukemia in 40 (33%). 96 patients (79%) had a central venous catheter (CVC), 94 patients (78%) were neutropenic. Blood cultures were positive for Enterobacteriaceae in 55 cases (45%), coagulase-negative staphylococci in 28 cases (23%), Group viridans Streptococcus in 19 (16%), Pseudomonas aeruginosa in 8 (7%). (9%). By the multivariate analysis, three factors remained significantly associated with length of stay of more than 14 days: CVC associated bacteremia (OR 21,73; CI95% 1.2-43.2; p 0.04), severe neutropenia (OR 1.75; CI95% 1.82-1.28; p 0.03) and coinfection (OR 27.4; CI95% 2.8-260.8; p 0.004). Conclusion: CVC associated bacteremia, severe neutropenia and viral coinfection were associated with hospital LOS of more than 14 days.

Trasplante de médula ósea alogénico en un paciente con fusariosis diseminada y leucemia mieloide aguda / Allogeneic hematopoietic cell transplantation in a patient with disseminated fusariosis and acute myeloid leukemia

La infección diseminada por Fusarium se ha convertido en un problema creciente en las personas con neoplasias hematológicas malignas, principalmente en pacientes con leucemias agudas; se describen cada vez más casos en aquellos sometidos a un trasplante de médula ósea. No existe un tratamiento óptimo establecido para la fusariosis diseminada. La mortalidad global comunicada de esta infección oscila entre el 50 y el 80%. Se presenta a continuación el caso de un paciente de sexo masculino de 29 años, con diagnóstico de leucemia mieloide aguda, que presenta como complicación una fusariosis diseminada, y logra sobrellevar un trasplante alogénico de médula ósea en el Hospital Italiano de San Justo (Argentina) de forma exitosa. (AU)

Disseminated fusariosis has become an increasing problem in people with hematopoietic neoplasms, mainly in patients affected by acute leukemias, and even more in those who undergo hematopoietic cell transplantation. There is not an optimal treatment for disseminated fusariosis. The global mortality described in the literature is between 50% and 80%. We introduce a case of a 29 year old patient with diagnosis of acute myeloid leukemia complicated with disseminated fusariosis, who copes with an allogeneic hematopoietic cell transplantation with a successful outcome in the "Hospital Italiano de San Justo" (Argentina). (AU)

Feohifomicosis nasal por Curvularia spicifera en un paciente pediátrico con neutropenia y leucemia mieloide aguda / Nasal phaeohyphomycosis by Curvularia spicifera in pediatric patient with neutropenia and acute myeloid leukemia

There are very few reports of pediatric patients with infections by dematiaceous filamentous fungi. In this publication we report a case of invasive fungal infection of the nasal septum by Curvularia spicifera in a pediatric patient with acute myeloid leukemia. The patient presented with a painful scabby wound in the nasal vestibule. Culture and universal PCR were consistent with Curvularia spicifera. Early management with surgical debridement and bi-associated antifungal therapy achieved complete resolution of the lesions, with no evidence of dissemination and relapses. Clinical management of these fungal infections represents a challenge as the antifungal selection and duration of therapy is not yet well stablished.

Existen pocos reportes de infecciones por hongos dematiáceos en pediatría. Comunicamos el caso de una infección fúngica invasora del tabique nasal en un niño con una leucemia mieloide aguda, que se presentó como una lesión costrosa dolorosa en el vestíbulo nasal. Se realizó desbridamiento quirúrgico precoz y recibió tratamiento antifúngico biasociado, lográndose resolución completa de las lesiones, sin diseminación ni recaídas. El cultivo y la RPC universal fueron compatibles con Curvularia spicifera. El manejo de estas infecciones fúngicas representa un desafío, considerando que la elección del agente antifúngico y la duración de la terapia no están completamente establecidas.

Diabetes insipidus como manifestação inicial de leucemia mieloide aguda em paciente com monossomia do cromossomo 7 / Diabetes insipidus as initial manifestation of acute myeloid leukemia in a patient with monosomy of chromosome 7

O diabetes insipidus (DI) central é uma síndrome caracterizada pela incapacidade de concentração urinária devido à deficiência do hormônio antidiurético. O envolvimento do sistema nervoso central é frequente nas leucemias, mas a ocorrência de DI é rara e confere pior prognóstico. A patogênese do DI na leucemia não é totalmente conhecida, mas a infiltração do eixo hipotálamo-hipofisário por células leucêmicas parece ser um fator responsável. O presente relato descreve o caso de um paciente que apresentou DI como primeira manifestação de leucemia mieloide aguda e que evoluiu com dificuldades de ajustes do sódio sérico, da poliúria e da reposição volêmica, necessitando de permanência prolongada em unidade de cuidados intensivos(AU)

Central diabetes insipidus (DI) is a syndrome characterized by the inability to concentrate urine due to a lack of antidiuretic hormone. Involvement of the central nervous system is common in acute leukemia, but the occurrence of DI is rare and determines a worse prognosis. The pathogenesis of DI in leukemia has not been fully understood yet, but infiltration of the hypothalamic-pituitary axis by leukemic cells seems to be involved. This report describes a case of a patient who presented with DI as the first manifestation of acute myeloid leukemia. Difficulties in the management of serum sodium, fluid replacement and polyuria led to prolonged length of stay in an intensive care unit(AU)

Mucormicosis rinosinusal por mucor hiemalis en una paciente con leucemia mieloide aguda / Rhinosinusal mucormycosis by mucor hiemalis in a patient with acute myeloid leukemia

Se reporta un caso clínico de una paciente femenina de 41 años, con antecedentes de leucemia mieloide aguda (LMA) en remisión. Estudiada por hematología, se confirmó recaída de LMA M4. Se inició quimioterapia. La paciente evolucionó con pancitopenia severa. Presentó dos episodios de neutropenia febril, el primero fue asociado a un absceso glúteo que se trató con antibacterianos, y el segundo a compromiso rinosinusal y úlcera necrótica de punta nasal, columela, tabique, cornete inferior izquierdo y paladar duro. Debido a la clínica e imá- genes radiológicas, se sospechó mucormicosis, por lo que se realizó cirugía con debridación extensa y se inició tratamiento antimicótico con anfotericina B desoxicolato. El cultivo de tejido informó abundante desarrollo de Mucor hiemalis. Se mantuvo pancitopénica durante aproximadamente un mes, siendo diariamente evaluada por un equipo multidisciplinario. Se hicieron varios aseos quirúrgicos, en el último se encontró tejido vital. La paciente completó diez días con anfotericina B desoxicolato y posteriormente se hizo traslape a posaconazol oral. Se realizó mielograma de control que evidenció remisión completa de recaída de LMA. Se dio de alta a su domicilio al día 40 de hospitalización, con hemograma adecuado y tratamiento oral con posaconazol para completar 6 semanas en total.

We report a case of a 41-years-old female patient with a history of acute myeloid leukemia (AML) in remission. Hematology studies confirmed relapse of AML M4. Chemotherapy was started. The patient developed severe pancytopenia. She presented two episodes of febrile neutropenia, the first one was associated with a gluteal abscess that was treated with antibacterials, and the second to rhinosinusal involvement and necrotic ulcer of nasal tip, columella, septum, left inferior turbinate and hard palate. Due to clinical and radiological imaging, mucormycosis was suspected, so surgery was performed with extensive debridement and antifungal treatment with amphotericin B deoxicholate was initiated. Tissue culture reported abundant development of Mucor hiemalis. She remained pancytopenic for approximately one month, being evaluated daily by a multidisciplinary team. Several surgical were made, finding vital tissue in the last perform. The patient completed ten days with amphotericin B deoxicholate and later was overlapped to oral posaconazole. A control myelogram was performed, showing complete remission of AML. She was discharged to her home at day 40 of hospitalization, with adequate blood count and oral treatment with posaconazole to complete 6 weeks in total.