Características del síndrome de lisis tumoral en pacientes pediátricos oncológicos. Un estudio observacional de centro único / Characteristics of tumor lysis syndrome in pediatric oncology patients. A single-center observational study

Introducción:El síndrome de lisis tumoral (SLT) es una emergencia oncológica, que produce alteraciones en el metabolismo, causando manifestaciones clínicas y trastornos bioquímicos que ponen en peligro la vida del paciente.El objetivo del presente estudio fue identificar las características clínicas, de laboratorio y tratamiento del SLT, en pacientes pediátricos onco-lógicos, del Instituto del Cáncer SOLCA-Cuenca, en el periodo 2010 ­2020.Materiales y métodos:En este estudio se identificó las características del SLT, en pacientes pediátricos oncológicos, del Instituto del Cáncer SOLCA-Cuenca, en el periodo 2010 ­2020, a través de un estudio de tipo descriptivo-observacional.Resultados:Seincluyó 463 historias clínicas, en el cual se obtuvo que el SLT tuvo una frecuen-cia del 5.61 %, con predominio del sexo masculino (57.7%) y con una edad media de 7 ± 1.29 años. La presentación clínica más observada fue la deshidratación con náusea, vómito y dia-rrea (57.7%). Las alteraciones de laboratorio más frecuentes fueron la hiperuricemia y la hi-pocalcemia, con un 76.9 %y un 73.1 %respectivamente. La Leucemia linfoblástica aguda (LLA) fue el diagnósticooncológico con más casos (61.5 %). Los pilares del tratamiento fue-ron la hiperhidratación y el uso de alopurinol, utilizados en el 100% y un 80.8 %respectiva-mente.Conclusión:El SLT afectó más frecuentemente a varones, con diagnóstico de leucemia, ma-nifestaciones clínicas digestivas y alteraciones de laboratorio (hiperuricemia e hipocalcemia). El tratamiento empleado resultó eficaz y se basó en lo recomendado por la literatura médica

Introduction:Tumor lysis syndrome (TLS) is an oncological emergency that results in meta-bolic alterations, causing clinical manifestations and biochemical disorders that endanger pa-tients' lives. The objective of the present study was to identify the clinical, laboratory, and treat-ment characteristics of TLSsin pediatric oncology patients at the SOLCA-Cuenca Cancer Ins-titute from 2010­2020.Materials and methods: In this study, the characteristics of TLS were identified in pediatric oncology patients at the SOLCA-Cuenca Cancer Institute from 2010 to 2020 through a des-criptive observational study.Results: A total of463 medical records were included. TLSs were associated witha frequency of 5.61%, with a predominance of males(57.7%) and a mean age of 7 ± 1.29 years. The most commonclinical presentation was dehydration with nausea, vomiting, and diarrhea (57.7%). The most frequent laboratory alterations were hyperuricemia and hypocalcemia, with 76.9% and 73.1%,respectively. The oncological diagnosis was acutelymphoblastic leukemia (ALL) in most patients(61.5%). The pillars of treatment were hyperhydration and allopurinol, used in 100% and 80.8%, respectively.Conclusion: TLSsmore frequently affectmen with a diagnosis of leukemia, digestive clinical manifestations, orlaboratory alterations (hyperuricemia and hypocalcemia). The treatment used was effective and based on what the medical literature recommended

Eficácia da rasburicase para prevenção e tratamento da síndrome de lise tumoral: uma revisão sistemática de literatura / Efficacy of rasburicase for prevention and treatment of tumor lysis syndrome: a systematic literature review / Eficacia de la rasburicasa en la prevención y tratamiento del síndrome de lisis tumoral: una revisión sistemática de la literatura

A síndrome de lise tumoral (SLT) é uma emergência onco-hematológica, associada à alta mortalidade e morbidade, que pode ocorrer espontaneamente ou em resposta à quimioterapia ou bioterapia anticâncer. A rasburicase é uma droga urato oxidase recombinante, a qual reduz o ácido úrico sanguíneo liberado, prevenindo e tratando a lesão renal aguda, que representa a principal complicação da SLT. O objetivo deste artigo foi avaliar a eficácia da rasburicase na prevenção e no tratamento da SLT, contribuindo para melhor compreensão do manejo dessa frequente síndrome em pacientes oncológicos. Foi realizada uma revisão de literatura sistematizada por meio de busca no banco de dados do PubMed e uptodate, de novembro de 2021 a janeiro de 2022, utilizando-se os descritores: prevention [title/abstract] AND prophylaxis [title/abstract] AND tumor lysis syndrome [title/abstract]. Dos 212 artigos encontrados, após exclusão por título, abstract e leitura completa, apenas nove foram selecionados. Os estudos mostraram, em sua maioria, uma redução do ácido úrico plasmático com o uso da rasburicase em pacientes com alto risco para SLT. A rasburicase foi eficaz para prevenção e tratamento da hiperuricemia em pacientes com risco de SLT. Apesar dos estudos analisados serem positivos para eficácia da rasburicase na prevenção e no tratamento da síndrome, nenhum deles trouxe como desfecho principal a redução de mortalidade. Torna-se relevante, portanto, a realização de mais estudos multicêntricos, prospectivos e com emprego de instrumentos validados sobre o tema desta revisão sistemática.

Tumor lysis syndrome (TLS) is an onco-hematological emergency associated with high mortality and morbidity, of spontaneous onset or in response to chemotherapy or anticancer biotherapy. Rasburicase is a recombinant urate oxidase drug that reduces blood uric acid released, preventing and treating acute kidney injury, considered the main TLS complication. This systematic literature review sought to evaluate the rasburicase effectiveness in preventing and treating tumor lysis syndrome, to better understand how to manage this frequent syndrome in cancer patients. Bibliographic search was conducted on the PubMed database from November 2021 to January 2022, using the following descriptors: prevention [title/abstract] AND prophylaxis [title/abstract] AND tumor lysis syndrome [title/abstract]. After exclusion by title, abstract and full reading, only nine papers were selected from the 212 found. Most studies showed reduced plasma uric acid by rasburicase use in high-risk patients for TLS. Rasburicase effectively prevented and treated hyperuricemia in patients at risk for tumor lysis syndrome. Despite these positive outcomes, none of the studies showed reduced mortality as the main outcome. Thus, further multicenter prospective studies using validated instruments are needed on the subject.

El síndrome de lisis tumoral (SLT) es una urgencia oncohematológica, asociada a una alta mortalidad y morbilidad, que puede presentarse de forma espontánea o en respuesta a quimioterapia o bioterapia anticancerígena. La rasburicasa es un fármaco de urato oxidasa recombinante, que reduce el ácido úrico sanguíneo liberado mediante la prevención y el tratamiento de la lesión renal aguda, que representa la principal complicación del SLT. El objetivo de este artículo fue evaluar la efectividad de la rasburicasa en la prevención y tratamiento del SLT, lo que contribuye a una mejor comprensión del manejo de este síndrome frecuente en pacientes oncológicos. Se hizo una revisión sistemática de la literatura mediante búsqueda en la base de datos PubMed y actualizada de noviembre de 2021 a enero de 2022, utilizando los descriptores de PubMed: prevention [title/abstract] AND prophylaxis [title/abstract] AND tumor lysis syndrome [title/abstract]. De los 212 artículos encontrados, después de la exclusión por título, resumen y lectura completa, solo 9 fueron seleccionados. La mayoría de los estudios mostraron una reducción del ácido úrico plasmático con el uso de rasburicasa en pacientes con alto riesgo de SLT. La rasburicasa fue eficaz para la prevención y el tratamiento de la hiperuricemia en pacientes con riesgo de síndrome de lisis tumoral. A pesar de que los estudios analizados fueron positivos para la eficacia de la rasburicasa en la prevención y tratamiento del síndrome, ninguno de ellos trajo como desenlace principal la reducción de la mortalidad. Por lo tanto, es relevante realizar más estudios prospectivos multicéntricos utilizando instrumentos validados sobre el tema de esta revisión sistemática.

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.

Generalidades de las principales urgencias en el paciente oncológico: revisión de tema / Overview of the main emergencies in cancer patients: Review / Generalidades das principais urgências no paciente oncológico: revisão de tema

Las urgencias oncológicas son complicaciones comunes de la evolución natural del tumor o de su manejo. Algunas pueden presentarse de manera sutil y ser pasadas por alto, lo que aumenta la morbimortalidad. El objetivo de esta revisión narrativa es recopilar información actualizada de las principales complicaciones oncológicas, para ello se realizó una revisión de artículos originales, revisiones sistemáticas y narrativas en bases de datos como Scopus, SciELO, PubMed, ScienceDirect y en el buscador Google Scholar. Se seleccionaron 63 referencias que mostraran información relevante acerca de las urgencias oncológicas planteadas para el desarrollo del artículo. En la revisión se discute que las complicaciones pueden clasificarse de acuerdo con su origen en infecciosas (neutropenia febril), metabólicas (síndrome de lisis tumoral e hipercalcemia maligna) y obstructivas (síndrome de vena cava superior, obstrucción intestinal, compresión medular y taponamiento cardiaco). El diagnóstico requiere un alto índice de sospecha, el médico debe tener la capacidad resolutiva y el conocimiento necesarios para el manejo y hacer uso racional de los recursos diagnósticos. Es necesario adoptar medidas terapéuticas que impacten positivamente en el pronóstico y que reduzcan la morbimortalidad.

Oncological emergencies are common complications resulting from the natural evolution of the tumor or its management; however, some of them may be subtle or even overlooked, which contributes to greater morbidity and mortality. Our aim was to gather updated information on the main oncological complications. A narrative literatura review was performed by searching for original articles, systematic reviews and narratives, in databases such as Scopus, SciELO, PubMed, ScienceDirect and in the Google Scholar search engine. 63 references were selected that addressed relevant information about the oncological emergencies raised for the development of the article. According to their origin, complications can be classified into infectious (febrile neutropenia), metabolic (tumor lysis syndrome and malignant hypercalcemia) and obstructive (superior vena cava syndrome, intestinal obstruction, spinal cord compression and cardiac tamponade). Facing these complications requires a high level of suspicion; the physician must be able to resolve each complication and have the necessary knowledge to approach each case, with a rational use of diagnostic resources. It is also necessary to adopt therapeutic measures that positively impact patients. patient prognosis, decreasing morbidity and death.

As urgências oncológicas são complicações comuns da evolução natural do tumor ou do seu manejo. Algumas podem apresentar-se de maneira sutil e ser passadaspor encima, o que aumenta a morbimortalidade. O objetivo desta revisão narrativa é recopilar informação atualizada das principais complicações oncológicas, para isso se realizou uma revisão de artigos originais, revisões sistemáticas e narrativas em bases de dados como Scopus, SciELO, PubMed, ScienceDirect e no buscador Google Scholar. Se selecionaram 63 referências que mostraram informação relevante sobre às urgências oncológicas apresentadas para o desenvolvimento do artigo. Na revisão se discuteque as complicações podem classificar-se de acordo com a sua origem em infecciosas (neutropenia febril), metabólicas (síndrome de lise tumoral e hipercalcemia maligna) e obstrutivas (síndrome de veia cava superior, obstrução intestinal, compressão medular e entupimento cardíaco). O diagnóstico requere um alto índice de suspeita, o médico deve ter a capacidade resolutiva e o conhecimento necessário para o manejo e fazer uso racional dos recursos diagnósticos. É necessário adotar medidas terapêuticas que impactem positivamente no prognóstico e que reduzam a morbimortalidade.

Síndrome de lisis tumoral secundario a la administración de corticoides en una adolescente sin enfermedad neoplásica confirmada: reporte de un caso / Tumor lysis syndrome after the administration of corticosteroids in an adolescent without confirmed neoplastic disease. Case report

El síndrome de lisis tumoral es una complicación potencialmente letal y constituye, junto con las infecciones, la emergencia oncológica más frecuente. En pediatría, este cuadro puede ser secundario a enfermedades neoplásicas, y los corticoides son un factor desencadenante. En este trabajo se presenta el caso de una paciente adolescente, sin neoplasias conocidas o evidentes, que desarrolló un síndrome de lisis tumoral luego de la administración de corticoides por sospecha de una infección respiratoria. Se discute la forma de presentación y los diagnósticos diferenciales del cuadro clínico inicial. Se hace especial foco en la administración de corticoides en cuadros clínicos en los que no existe evidencia científica que respalde fuertemente su indicación. El uso de corticosteroides sistémicos en infecciones respiratorias agudas debe ser evaluado en el contexto clínico y solo debe indicarse en situaciones con probada efectividad.

Tumor lysis syndrome is a potentially lethal complication and constitutes with infections the most frequent oncological emergency. In children, this condition can be secondary to neoplastic diseases, with corticosteroids being a triggering factor. This paper presents the case of an adolescent patient, without known or obvious neoplasms, who developed a tumor lysis syndrome after the administration of corticosteroids due to suspected respiratory infection.The clinical presentation and differential diagnoses are discussed. Special focus is placed on the administration of corticosteroids in clinical conditions with weak scientific evidence. The use of systemic corticosteroids in acute respiratory infections should be evaluated in the clinical context and only indicated in situations with proven effectiveness.

Efficacy of low-dose uric acid oxidase in the treatment of 106 pediatric patients with aggressive mature B-cell non-Hodgkin lymphoma and hyperuricemia / 中华实用儿科临床杂志

Objective:To investigate the efficacy of low-dose uric acid oxidase in treating children with aggressive mature B-cell non-Hodgkin lymphoma accompanied by hyperuricemia.Methods:Clinical data of children with primary aggressive mature B-cell non-Hodgkin lymphoma and hyperuricemia, who were treated in Beijing Children′s Hospital, Capital Medical University from January 2016 to June 2021 were retrospectively analyzed.The serum uric acid concentration was monitored in all pediatric patients from the day before chemotherapy to the seventh day of chemotherapy.Low-dose uric acid oxidase [0.05-0.10 mg/(kg·dose)] was intravenously injected into the patients when the serum uric acid level exceeded the upper limit of the normal range.The therapeutic effect and clinical medication experience of uric acid oxidase were summarized.The change of serum uric acid levels with time before and after the application of different doses of uric acid oxidase was analyzed by a repeated measures ANOVA. Results:A total of 106 children with primary aggressive mature B-cell non-Hodgkin lymphoma and hyperuricemia were enrolled in this study.There were 88 males and 18 females, with a median age of 6.5 (3.5, 10.0) years.The pathological subtypes comprised Burkitt′s lymphoma in 95 cases (89.6%), high-grade B-cell lymphoma in 7 cases (6.6%), and diffuse large B-cell lymphoma in 4 cases (3.8%). Additionally, 39 cases (36.8%) were in clinical stage Ⅲ and 67 cases (63.2%) were in stage Ⅳ.All cases had high tumor burden, including renal involvement in 52 cases (49.1%), tumor lysis syndrome in 42 cases (39.6%), and acute kidney injury in 27 cases (25.5%). Totally, one dose of uric acid oxidase was intravenously injected into 41 children (38.7%), 41 children (38.7%) were given 2 dosages, 20 children (18.9%) were given 3 dosages, and 4 children (3.8%) received 4 dosages.Moreover, 9 cases (8.5%) were supplemented with continuous renal replacement therapy.Serum uric acid concentrations before chemotherapy and 12 hours after injecting the first dose of uric acid oxidase were (741.4±312.9) μmol/L and (210.8±148.6) μmol/L, respectively.The difference was statistically significant ( t=5.288, P<0.001). The change of serum uric acid levels over time before and after the application of different doses of uric acid oxidase in children was compared, and no significant difference was found ( F=0.225, P=0.879). No delay in chemotherapy or death arising from tumor lysis syndrome and acute kidney injury occurred within 28 days after chemotherapy. Conclusions:Low-dose and on-demand application of uric acid oxidase can rapidly and effectively reduce serum uric acid levels in children with aggressive mature B-cell non-Hodgkin lymphoma in the early stage of chemotherapy.

Efficacy of low-dose uric acid oxidase in the treatment of 106 pediatric patients with aggressive mature B-cell non-Hodgkin lymphoma and hyperuricemia / 中华实用儿科临床杂志

Objective:To investigate the efficacy of low-dose uric acid oxidase in treating children with aggressive mature B-cell non-Hodgkin lymphoma accompanied by hyperuricemia.Methods:Clinical data of children with primary aggressive mature B-cell non-Hodgkin lymphoma and hyperuricemia, who were treated in Beijing Children′s Hospital, Capital Medical University from January 2016 to June 2021 were retrospectively analyzed.The serum uric acid concentration was monitored in all pediatric patients from the day before chemotherapy to the seventh day of chemotherapy.Low-dose uric acid oxidase [0.05-0.10 mg/(kg·dose)] was intravenously injected into the patients when the serum uric acid level exceeded the upper limit of the normal range.The therapeutic effect and clinical medication experience of uric acid oxidase were summarized.The change of serum uric acid levels with time before and after the application of different doses of uric acid oxidase was analyzed by a repeated measures ANOVA. Results:A total of 106 children with primary aggressive mature B-cell non-Hodgkin lymphoma and hyperuricemia were enrolled in this study.There were 88 males and 18 females, with a median age of 6.5 (3.5, 10.0) years.The pathological subtypes comprised Burkitt′s lymphoma in 95 cases (89.6%), high-grade B-cell lymphoma in 7 cases (6.6%), and diffuse large B-cell lymphoma in 4 cases (3.8%). Additionally, 39 cases (36.8%) were in clinical stage Ⅲ and 67 cases (63.2%) were in stage Ⅳ.All cases had high tumor burden, including renal involvement in 52 cases (49.1%), tumor lysis syndrome in 42 cases (39.6%), and acute kidney injury in 27 cases (25.5%). Totally, one dose of uric acid oxidase was intravenously injected into 41 children (38.7%), 41 children (38.7%) were given 2 dosages, 20 children (18.9%) were given 3 dosages, and 4 children (3.8%) received 4 dosages.Moreover, 9 cases (8.5%) were supplemented with continuous renal replacement therapy.Serum uric acid concentrations before chemotherapy and 12 hours after injecting the first dose of uric acid oxidase were (741.4±312.9) μmol/L and (210.8±148.6) μmol/L, respectively.The difference was statistically significant ( t=5.288, P<0.001). The change of serum uric acid levels over time before and after the application of different doses of uric acid oxidase in children was compared, and no significant difference was found ( F=0.225, P=0.879). No delay in chemotherapy or death arising from tumor lysis syndrome and acute kidney injury occurred within 28 days after chemotherapy. Conclusions:Low-dose and on-demand application of uric acid oxidase can rapidly and effectively reduce serum uric acid levels in children with aggressive mature B-cell non-Hodgkin lymphoma in the early stage of chemotherapy.

Fatal tumor lysis syndrome in a patient with metastatic gastric adenocarcinoma

Tumor lysis syndrome is a well-characterized and potentially deadly complication of spontaneous or treatment-related tumor destruction, and it is most commonly associated with hematologic malignancies. Our case illustrates a rare example of fatal tumor lysis syndrome in the setting of metastatic gastric adenocarcinoma treated with radiation therapy. This case highlights the critical importance of identifying patients with solid organ malignancies at risk for tumor lysis syndrome and of early recognition and treatment of this syndrome.

Lesão Renal Aguda por Síndrome da Lise Tumoral em Unidade de Terapia Intensiva- Conhecimento do Enfermeiro / Acute Kidney Injury due to Tumor Lysis Syndrome in the Intensive Care Unit - Nurses Knowledge / Lesión renal aguda por síndrome de lisis tumoral en la Unidad de Cuidados Intensivos - Conocimiento de las enfermeras

Objetivo: analisar a produção científica sobre a lesão renal aguda causada pela síndrome da Lise tumoral no paciente internado em unidade de terapia intensiva, bem como o conhecimento do enfermeiro sobre tal patologia. Método: Trata-se de um artigo de revisão integrativa realizado através da leitura de 30 artigos científicos retirados da Biblioteca Virtual de Saúde. Resultados: Identificouse a ocorrência da lesão renal aguda e síndrome de lise tumoral através das alterações metabólicas e hemodinâmicas nos pacientes internados na unidade de terapia intensiva e observou-se que não há publicações com relatos do enfermeiro sobre o conhecimento desta patologia. Conclusão: Apesar dos estudos atuais e a busca constante pelo conhecimento, sabe-se que a lesão renal aguda e Síndrome de Lise Tumoral é uma emergência oncológica com alta taxa de morbidade, onde a principal estratégia para melhorar a evolução de pacientes é estabelecer medidas profiláticas e o tratamento adequado com urgência. Deve existir uma análise contínua do enfermeiro, bem como de toda equipe, estratificação dos riscos e elaboração de protocolos de controles hidroeletrolíticos e laboratoriais para estabilização hemodinâmica do paciente oncológico na unidade de terapia intensiva.

Objective: to analyze the scientific production on acute kidney injury caused by tumor lysis syndrome in patients admitted to the intensive care unit, as well as the nurses' knowledge about such pathology. Method: This is an integrative review article carried out by reading 30 scientific articles taken from the Virtual Health Library. Results: The occurrence of acute kidney injury and tumor lysis syndrome was identified through metabolic and hemodynamic changes in patients admitted to the intensive care unit and it was observed that there are no publications with nurses' reports on the knowledge of this pathology. Conclusion: Despite current studies and the constant search for knowledge, it is known that acute kidney injury and Tumor Lysis Syndrome is an oncological emergency with a high morbidity rate, where the main strategy to improve the evolution of patients is to establish prophylactic measures and appropriate urgent treatment. There must be a continuous analysis of the nurse, as well as the entire team, risk stratification and elaboration of hydroelectrolytic and laboratory control protocols for hemodynamic stabilization of the cancer patient in the intensive care unit.

Objetivo: analizar la producción científica sobre la lesión renal aguda por síndrome de lisis tumoral en pacientes ingresados en la unidad de cuidados intensivos, así como el conocimiento de los enfermeros sobre dicha patología. Método: Se trata de un artículo de revisión integradora realizada mediante la lectura de 30 artículos científicos extraídos de la Biblioteca Virtual en Salud. Resultados: Se identificó la ocurrencia de daño renal agudo y síndrome de lisis tumoral a través de cambios metabólicos y hemodinámicos en pacientes ingresó en la unidad de cuidados intensivos y se observó que no existen publicaciones con informes de enfermeras sobre el conocimiento de esta patología. Conclusión: a pesar de los estudios actuales y la búsqueda constante de conocimiento, se sabe que la lesión renal aguda y el síndrome de lisis tumoral es una emergencia oncológica con una alta morbilidad, donde la principal estrategia para mejorar la evolución de los pacientes es establecer medidas profilácticas. y tratamiento urgente apropiado. Se debe realizar un análisis continuo de la enfermera, así como de todo el equipo, estratificación de riesgo y elaboración de protocolos de control hidroelectrolítico y de laboratorio para la estabilización hemodinámica del paciente oncológico en la unidad de cuidados intensivos.

Tumor lysis syndrome in multiple myeloma treated by daratumumab combined with chemotherapy: report of two cases and review of literature / 白血病·淋巴瘤

Objective:To investigate the clinical safety of daratumumab (DARA) in patients with relapsed/refractory multiple myeloma (MM).Methods:The diagnosis and treatment process of 2 cases of relapsed/refractory MM treated in Huadong Hospital of Fudan University in 2020 were retrospectively analyzed, the related laboratory indicators indexes after using DARA were analyzed, and the related literature was reviewed.Results:After using DARA, the 2 patients rapidly developed tumor lysis syndrome (TLS) such as high potassium, high phosphorus, high uric acid, low calcium and kidney damage, which eventually led to the death of patients.Conclusions:Patients with high tumor load treated by DARA are prone to TLS, which is often life-threatening. Clinicians should be alert to the occurrence of such events.

Recomendaciones para el manejo de la lisis tumoral / Recommendations for tumor lysis syndrome management

El síndrome de lisis tumoral representa una complicación potencialmente letal provocada por la liberación masiva de ácidos nucleicos, potasio y fosfato hacia la circulación como resultado de la lisis de células neoplásicas, las cuales se caracterizan por una rápida capacidad de proliferación y alta sensibilidad a fármacos. Esto puede ocurrir de forma espontánea antes del inicio del tratamiento y agravarse luego de haberse iniciado la quimioterapia. Presenta una alta mortalidad. Su prevención continúa siendo la medida terapéutica más importante. El cuadro clínico se caracteriza por la existencia de trastornos del metabolismo hidroelectrolítico, en particular, hipercalemia, hiperfosfatemia e hiperuricemia y por la aparición de una lesión renal aguda. Una adecuada intervención terapéutica implica hidratación intravenosa y medidas para prevenir o corregir las alteraciones metabólicas. En este artículo, se proponen lineamientos para seguir tanto en la etapa diagnóstica como en el tratamiento de esta complicación.

The tumor lysis syndrome represents a potentially lethal complication caused by the massive release of nucleic acids, potassium and phosphate into the circulation as a result of the lysis of neoplastic cells, which are characterized by a rapid proliferation capacity and high sensitivity to drugs. This may occur spontaneously prior to the start of treatment, becoming worse after the initiation of chemotherapy. It presents a high mortality; its prevention continues being the most important therapeutic measure. The clinical picture is characterized by the existence of hydroelectrolytic metabolism disorders, in particular hyperkalemia, hyperphosphatemia and hyperuricemia and by the appearance of an acute renal lesion. Adequate therapeutic intervention involves intravenous hydration and measures to prevent or correct metabolic alterations. This article proposes guidelines to follow both in the diagnostic stage and in the treatment of this complication.

Metastatic adult neuroblastoma with spontaneous tumor lysis syndrome

Neuroblastoma (NB) is a solid tumor of the sympathetic nervous system, most commonly found in childhood, standing for 7% of all pediatric malignancies. The incidence in adults is markedly smaller: 1 case per 10 million adults per year. We report the case of a previously healthy 27-year-old woman who started with lumbar pain, asthenia, and abdominal distension over the last month. A chest and abdomen tomography scan showed a huge mass in the upper left hemithorax and marked hepatomegaly. The diagnosis was confirmed by hepatic and lung biopsies. On day 4, after admission, the patient started chemotherapy. On the following days, she had severe vaginal bleeding, epistaxis, worsening of the hepatic function markers, refractory shock, and multiple organ dysfunction. She died on the twelfth day of admission. We also present a review of adult cases of NB reported in the past 5 years.

A Case of Oxycodone-related Respiratory Depression Induced by Tumor Lysis Syndrome

Tumor lysis syndrome (TLS) is a metabolic disorder that is caused by acute lysis of massive tumor cells. We report a case with opioids-related severe respiratory depression induced by TLS. A 39-year-old man received chemotherapy for mycosis fungoides. Two hours after administration of chemotherapeutic agents, his renal function worsened, and he was diagnosed with TLS by laboratory and clinical findings. Moreover, he showed severe respiratory depression and pinpoint pupils, and become drowsy. These symptoms were attributed to oxycodone that had been administered to treat his tumor-related cutaneous pain, and were improved by injection of anti-opioids agent naloxone. In this case, we consider that the clearance of oxycodone was disrupted by renal dysfunction caused by TLS, leading to enhancement of the effects of oxycodone.

Síndrome de lisis tumoral / Tumor lysis syndrome

El síndrome de lisis tumoral (SLT) es una entidad poco frecuente y potencialmente fatal. Representa una emergencia oncológica. Puede diagnosticarse por su forma de presentación clínica y también por los resultados de laboratorio. En la mayoría de los casos se presenta como complicación del tratamiento quimioterapéutico de enfermedades oncohematológicas con gran masa tumoral. Con menor frecuencia se ha descrito un síndrome de lisis tumoral espontáneo, o secundario al uso de corticoides, hidroxiurea y radioterapia. En sus formas más graves puede requerir internación en unidades de terapia intensiva y medidas terapéuticas invasivas como la hemodiálisis. Comunicamos cuatro casos de SLT con características de presentación inusual internados en nuestro Instituto de Investigaciones Médicas.

Tumor lysis syndrome (SLT) is a rare and potentially fatal entity. It represents an oncological emergency. It can be diagnosed by its clinical presentation and also by laboratory results. In most cases it is presented as a complication of the chemotherapeutic treatment of oncohematological diseases with large tumor mass. Less frequently, a syndrome of spontaneous tumor lysis has been described, or secondary to the use of corticosteroids, hydroxyurea and radiotherapy. In its most severe forms it may require hospitalization in intensive care units and invasive therapeutic measures such as hemodialysis. We report four cases of SLT with unusual presentation characteristics admitted to our Medical Research Institute.

Transient leukaemia of Down syndrome in a neonate: case report

Transient leukemia of Down syndrome(TL-DS)' or transient myeloproliferative disorder (TMD) or transient abnormal myelopoiesis (TAM) is a hematologic abnormality characterized by an uncontrolled proliferation of myeloblasts in peripheral blood and bone marrow which characteristically affects newborns and babies with Down syndrome. Children with Down syndrome (Trisomy 21) have a unique predisposition to develop myeloid leukemia of Down syndrome(ML-DS). In majority of cases of TL-DS, the GATA1 mutant clone goes into spontaneous remission without the need for chemotherapy. However, 10-20 % of neonates with TL-DS and silent TL-DS subsequently develop ML-DS in the first 5 years of life due to additional oncogenic mutations acquired by the persistent GATA1 mutant cells. We present here, one such case of Down syndrome with TL-DS in a neonate.

Tumor Lysis Syndrome in Hematological Malignancies Presenting at a Tertiary Care Hospital in Pakistan

Background: Tumor lysis syndrome (TLS) is a group of metabolic derangements after the malignant cells die with treatment and leads to complications such as acute renal failure, cardiac arrhythmias, seizures, multiple organ failure, and sudden death. TLS is a common potentially preventable complication of hematological malignancies which are the most common cancers in our province. But the data about frequent complications in the course of their management such as TLS is rudimentary. Objective: The main objective of this study was to determine how frequently TLS occurs in our patients. Design and methods: A descriptive cross-sectional study was designed and conducted in Pathology Department, King Edward Medical University, Lahore (February 2014-July 2014). Newly diagnosed patients of hematological malignancies were enrolled in the study. The clinical parameters such as age, gender and laboratory parameters such as laboratory diagnosis, Complete Blood Count, Serum Potassium, Serum LDH, Serum Phosphate, Serum Uric Acid, Serum Calcium, and Serum Creatinine were evaluated. Results: A total of 130 patients were enrolled in the study. Eighty were males and 50 were females. Mean age was 47.02 ± 15 years. Thirtytwo patients (25%) fulfilled the criteria for TLS in our setting. TLS was twice more common in females and in 61-80 year age group (36.67%). The frequency of TLS in each hematological malignancy was as follows: ALL 6.15%, AML 5.38%, NHL 5.38%, CML 4.62%, CLL 2.31% and HD 0.77%. Conclusion: TLS is not an uncommon complication of hematological malignancies in our part of the world. It usually occurs after treatment and can be diagnosed and monitored by routinely available biochemical tests. A high index of suspicion is required to optimize the oncology care as this can adversely affect the clinical outcome of these patients

The treatment of tumor dissolved syndrome / 中国小儿急救医学

Tumor lysis syndrome is an oncologic emergency that seriously threatens the lives of pa-tients. Prevention of tumor lysis syndrome is more important than treatment. According to the risk classifica-tion,identifying high risk patients and effective preventive measures can improve the prognosis of patients. Patients with tumor lysis syndrome should receive hydration, rasburicase, diuretics, correction of electrolyte imbalances and appropriate renal replacement therapy.

Pathogenesis and diagnosis of tumor lysis syndrome / 中国小儿急救医学

Tumor lysis syndrome( TLS) is an emergency in hematology and oncology department. It is a serious or fatal complication of malignancy patients. This article summarized its definition, etiology, pathogenesis and diagnosis. Early identification of its clinical manifestations,familiar with the risk factors and severity grading leading to TLS,and targeted early prevention and treatment can save the lives of the patients with TLS.

Síndrome de lise tumoral / Tumor lysis syndrome

Introdução: A expansão das opções terapêuticas para pacientes oncológicos nos últimos anos leva ao aumento na probabilidade de intercorrências ao longo do tratamento. Portanto, as emergências oncológicas têm se tornado ainda mais frequentes. A síndrome de lise tumoral (SLT) é uma emergência oncológica de grande importância clínica que merece atenção de todo médico. Métodos: este capítulo é uma revisão dos artigos sobre SLT das principais revistas de clínica médica da biblioteca virtual MEDLINE e é um protocolo baseado nos guidelines internacionais mais recentes. Resultados: A SLT ocorre a partir da lise das células tumorais e dos distúrbios metabólicos associados ao conteúdo intracelular extravasado, que resultam em alterações laboratoriais metabólicas e clínicas. Para manejo adequado dos pacientes de risco, deve ser feita a estratificação desses pacientes em baixo, intermediário e alto risco de desenvolvimento de SLT. Conclusão: O principal aspecto para êxito no manejo de pacientes com SLT aguda é manter um alto índice de suspeição e de vigilância, identificando pacientes de alto risco e instituindo hidratação e terapia medicamentosa imediatamente.

Introduction: The expansion on therapeutic possibilities for oncologic patients in the last years leads to an increase on the probability of intercurrences along the clinical course. Therefore, oncologic emergencies are becoming more frequent. Tumor Lysis Syndrome (TLS) is an oncologic emergency of great clinical importance that deserves every physician's attention. Methods: This chapter is a review of the major clinical articles on Medline Database Regarding TLS and a protocol based on the most recent international guidelines. Results: Once tumor cells experience lysis, discharge of intracellular content can cause different metabolic disturbances, that result in laboratory disorders or, even further, in clinical presentation. Conclusion: The key aspect on management of acute TLS is maintaining high clinical suspicion and surveillance to identify patients on high risk of developing TLS and to establish hydration and pharmacological therapy immediately.

A Lethal Case of Tumor Lysis Syndrome in Hypopharyngeal Cancer

<p>Tumor lysis syndrome (TLS) is an oncological emergency characterized by various metabolic abnormalities, such as hyperuricemia, hyperphosphatemia, hyperkalemia and hypocalcemia. Although TLS is rare in solid tumors, it has been reported in various cancers and sarcomas. It can be caused by chemotherapy, radiotherapy, surgical procedures. TLS in solid tumors may be lethal when it once develops. Hence, prophylaxis is considered important in medium risk diseases. We experienced the case that serum lactate dehydrogenase (LDH) level had elevated drastically a month prior to the onset of TLS in hypopharyngeal cancer. It is suggested that monitoring of LDH may be helpful for predicting the onset of TLS.</p>