Elements for assistance to patients with hematological malignancies to propose care lines: a scoping review.

OBJECTIVES: to identify the elements for assistance to patients with hematological malignancies to propose a care line. METHODS: this is a scoping review, anchored in the JBI theoretical framework, with searches carried out in April 2021, in eight electronic databases and 10 repositories of theses and dissertations. RESULTS: the final sample consisted of 93 studies, and the main forms of assistance provided that can support a care line for this public were imaging tests, immunophenotyping, chemotherapy regimens, radiotherapy, infection management, assessment of nutritional status, maintenance of oral function, symptom management and screening for second malignancies. CONCLUSIONS: the elaboration of a care line for onco-hematologic patients is necessary, considering the complexity surrounding the diagnosis and treatment of hematologic malignancies, in addition to the difficulties that are imposed in relation to access and continuity of care in the network.

Elements for assistance to patients with hematological malignancies to propose care lines: a scoping review / Elementos de asistencia a pacientes con neoplasias hematológicas para proponer líneas de atención: revisión de alcance / Elementos para assistência a pacientes com neoplasias hematológicas para propor linhas de cuidado: scoping review

ABSTRACT Objectives: to identify the elements for assistance to patients with hematological malignancies to propose a care line. Methods: this is a scoping review, anchored in the JBI theoretical framework, with searches carried out in April 2021, in eight electronic databases and 10 repositories of theses and dissertations. Results: the final sample consisted of 93 studies, and the main forms of assistance provided that can support a care line for this public were imaging tests, immunophenotyping, chemotherapy regimens, radiotherapy, infection management, assessment of nutritional status, maintenance of oral function, symptom management and screening for second malignancies. Conclusions: the elaboration of a care line for onco-hematologic patients is necessary, considering the complexity surrounding the diagnosis and treatment of hematologic malignancies, in addition to the difficulties that are imposed in relation to access and continuity of care in the network.

RESUMEN Objetivos: identificar los elementos para la asistencia a pacientes con neoplasias hematológicas para proponer una línea de atención. Métodos: se trata de una revisión de alcance, anclada en el marco teórico del JBI, con búsquedas realizadas en abril de 2021 en ocho bases de datos electrónicas y 10 repositorios de tesis y disertaciones. Resultados: la muestra final estuvo compuesta por 93 estudios, y las principales formas de asistencia brindadas que pueden sustentar una línea de atención a este público fueron pruebas de imagen, inmunofenotipificación, regímenes de quimioterapia, radioterapia, manejo de infecciones, evaluación del estado nutricional, mantenimiento de la función oral, manejo de síntomas y detección de segundas neoplasias malignas. Conclusiones: es necesario el desarrollo de una línea de atención al paciente oncohematológico, dada la complejidad que rodea al diagnóstico y tratamiento de las neoplasias hematológicas, además de las dificultades que se imponen en relación al acceso y continuidad de la atención en una red.

RESUMO Objetivos: identificar os elementos para assistência a pacientes com neoplasias hematológicas para propor uma linha de cuidado. Métodos: trata-se de uma scoping review, ancorada no referencial teórico do JBI, com buscas realizadas em abril de 2021 em oito bases de dados eletrônicas e 10 repositórios de teses e dissertações. Resultados: a amostra final foi composta por 93 estudos, e as principais formas de assistências prestadas que podem embasar uma linha de cuidado para esse público foram exames de imagem, imunofenotipagem, regimes quimioterápicos, radioterapia, gestão de infecções, avaliação do estado nutricional, manutenção da função oral, gerenciamento de sintomas e rastreio para segundas neoplasias. Conclusões: a elaboração de uma linha de cuidados para pacientes onco-hematológicos se faz necessária, tendo em vista a complexidade que cerca o diagnóstico e tratamento das neoplasias hematológicas, além das dificuldades que se impõem em relação ao acesso e continuidade do cuidado em rede.

Current global scenario of guidelines on the management of paroxysmal nocturnal hemoglobinuria: a systematic literature review / Cenário global de diretrizes para o manejo da hemoglobinúria paroxística noturna: uma revisão sistemática da literatura

Objective: To report the outcomes of a systematic literature review of guidelines and consensus on the management of paroxysmal nocturnal hemoglobinuria (PNH) and describe the main therapeutic options available worldwide. Methods: A systematic literature review was conducted in April 2018 with no time limit and reported in line with the PRISMA statement. The AGREE II instrument was used to determine the quality of each guideline included in the systematic review. Results: Eight guidelines/consensus were eligible, one developed by an international group, two in Spain, and one each in Turkey, Germany, Argentina, Australia and the United Kingdom. Supportive treatment with erythrocyte transfusion, anticoagulants and steroids is indicated by all guidelines and consensus. The use of erythropoietin is suggested by three of them. Recommendations for the prescription of eculizumab were consistent in all but one guideline, published in 2005. Allogeneic hematopoietic stem cell transplantation is reported as the only potentially curative treatment for PNH, although its association with high mortality and morbidity rates is emphasized, being indicated for a selected group of patients. The AGREE II scores applied for each domain showed in general a low and heterogeneous methodological quality among guidelines. Conclusion: Despite the low and heterogeneous methodological quality, in general the comparison of guidelines and consensus for PNH management showed consistent recommendations regarding supportive care, eculizumab and hematopoietic stem cell transplantation.

Objetivo: Relatar os desfechos de uma revisão sistemática da literatura de diretrizes e documentos de consenso sobre o manejo da hemoglobinúria paroxística noturna (HPN) e descrever as principais opções terapêuticas disponíveis mundialmente. Métodos: Uma revisão sistemática da literatura foi conduzida em abril de 2018 sem limite temporal e realizada de acordo com a recomendação PRISMA. O instrumento AGREE II foi utilizado para determinar a qualidade de cada diretriz incluída na revisão. Resultados: Foram elegíveis oito diretrizes/consensos, um desenvolvido por um grupo internacional, dois na Espanha e um em cada um dos países a seguir: Turquia, Alemanha, Argentina, Austrália e Reino Unido. O tratamento de suporte com transfusão de eritrócitos, anticoagulantes e esteroides é indicado por todos os documentos. A eritropoetina é indicada por três deles. A recomendação de prescrição do eculizumabe foi consistente em todos, exceto em um publicado em 2005. O transplante alogênico de células-tronco hematopoéticas é reportado como o único tratamento com potencial curativo para a HPN, apesar de uma enfática associação com maiores taxas de mortalidade e morbidade, sendo indicado para grupos selecionados de pacientes. Os escores AGREE II aplicados para cada domínio demonstraram, em geral, qualidade metodológica baixa e heterogênea entre as diretrizes. Conclusão: Apesar da qualidade metodológica baixa e heterogênea, em geral, a comparação de diretrizes e consensos para o manejo da HPN demonstrou recomendações consistentes quanto ao uso de tratamento de suporte, eculizumabe e transplante alogênico de células-tronco hematopoiéticas.

Invasive fungal infection by Cryptococcus neoformans var. grubii with bone marrow and meningeal involvement in a HIV-infected patient: a case report.

BACKGROUND: Cryptococcosis is a common opportunistic infection in patients infected by Human Immunodeficiency Virus (HIV) and is the second leading cause of mortality in Acquired Immunodeficiency Syndrome (AIDS) patients worldwide. The most frequent presentation of cryptococcal infection is subacute meningitis, especially in patients with a CD4+ T Lymphocytes count below 100 cells/µL. However, in severely immunosuppressed individuals Cryptococcus neoformans can infect virtually any human organ, including the bone marrow, which is a rare presentation of cryptococcosis. CASE PRESENTATION: A 45-year-old HIV-infected male patient with a CD4+ T lymphocyte count of 26 cells/µL who presented to the emergency department with fever and pancytopenia. Throughout the diagnostic evaluation, the bone marrow aspirate culture yielded encapsulated yeasts in budding, identified as Cryptococcus sp. The bone marrow biopsy revealed a hypocellularity for age and absence of fibrosis. It was observed presence of loosely formed granuloma composed of multinucleated giant cells encompassing rounded yeast like organisms stained with mucicarmine, compatible with Cryptococcus sp. Then, the patient underwent a lumbar puncture to investigate meningitis, although he had no neurological symptoms and neurological examination was normal. The cerebrospinal fluid culture yielded Cryptococcus sp. The species and genotype identification step showed the infection was caused by Cryptococcus neoformans var. grubii (genotype VNI). The patient was initially treated with amphotericin B deoxycholate plus fluconazole for disseminated cryptococcosis, according to guideline recommendations. However, the patient developed acute kidney injury and the treatment was switched for fluconazole monotherapy. The symptoms disappeared completely with recovery of white blood cells and platelets counts. Cerebrospinal fluid cultures for fungi at one and two-weeks of treatment were negative. CONCLUSIONS: Bone marrow infection caused by Cryptococcus neoformans is a rare presentation of cryptococcosis. The cryptococcal infection should be included for differential diagnosis in HIV-infected patients with fever and cytopenias, especially when CD4+ T lymphocytes count is below 100 cells/µL.