Tumor lysis syndrome in hematological inpatients, experience from a university hospital in Brazil: A retrospective cohort study.

INTRODUCTION: The tumor lysis syndrome is a medical emergency. Its presentation can be spontaneous or secondary, as a consequence of the established treatment. The diagnosis and treatment of the tumor lysis syndrome (TLS) has become a crucial goal to the evolution and improvement of treatments and targeted therapies. METHODS: Between January 2014 and December 2019, we retrospectively reviewed inpatients aged over 18 years with hematological neoplasms from a tertiary hospital in Brazil. We identified 112 episodes of TLS in 97 patients. The incidence was 10.5% and the median OS was 13.0 months (95%CI 6.2 - 19.7). The median age was 56 years (IQR 39.5 - 64). The most frequent diagnoses were multiple myeloma (18.6%), acute myeloid leukemia (17.5%) and diffuse large B-cell lymphoma (17.5%). All patients received intravenous (IV) hydration. The management also included the administration of allopurinol in 76% of the cases and rasburicase in eight patients. Renal replacement therapy was necessary in 37% of the cases. In the multivariate analysis, age, HIV status and ICU treatment were significantly associated with OS. CONCLUSION: The TLS is a serious complication in the setting of hematological malignancies. The use of scores for risk stratification, as well as the inclusion of prophylactic measures and prompt treatment with frequent laboratory monitoring, is essential to reduce morbidity and mortality in the comprehensive treatment of these patients.

Prophylaxis in hemophilia

Hemophilia is an inherited X-linked coagulopathy defined by a deficiency or abnormality in the clotting function of factor VIII (Hemophilia A) or factor IX (Hemophilia B). Prophylaxis ­ the regular administration of therapeutic products to maintain hemostasis and prevent bleeding ­ is the mainstream of treatment. Addressing the development and scientific evidence for administrating prophylaxis is the goal of this review. Prophylaxis is the therapeutic modality of choice for people with severe hemophilia, being considered, in principle, a lifelong treatment. It should have an early onset, ideally as a primary, or at least secondary. Even lifelong tertiary prophylaxis seems to offer benefit, although further studies are still lacking. Individualized strategies should lead to an optimization of the dilemma between better joint outcomes versus involved costs.

Hemophilia: a biography on therapeutical approaches

The history of hemophilia is ancient, with descriptions dated to the 2nd century AD. The first modern narratives appeared in 1800s, when total blood transfusion was the only available treatment and life expectancy was remarkably low. Advances occurred with the use of plasma and cryoprecipitate, but only the discovered of factor concentrates revolutionized the treatment. The implantation of prophylaxis allowed hemophilic patients to prevent bleeding and the development of chronic arthropathy, although with a significant burdensome with the regular infusions. In the past 20 years, this field has witnessed major improvements, including the development of gene therapy and other pharmacological approaches.

Malignant pleural effusion in multiple myeloma: a case report / Derrame pleural malign em mieloma múltiplo: relato de caso

Multiple myeloma (MM) is a malignant neoplasm of monoclonal plasma cells that accumulate in bone marrow (BM). Malignant pleural effusions (MPE), as part of multiple myeloma clinical presentation, are unusual. Is even more rare as the first sign of presentation, occurring in less than 1% of the cases. The most common associated immunoglobulin with malignant pleural effusions is IgA subtype (80%). This condition carry a poor prognosis. We aim to describe a refractory case of multiple myeloma with extensive disease that presented with extramedullary relapse with malignant pleural effusions , besides discussing the importance of differential diagnosis.

O mieloma múltiplo (MM) é uma neoplasia maligna de células plasmáticas monoclonais que se acumulam na medula óssea (MO). Os derrames pleurais malignos (EPM), como parte da apresentação clínica do mieloma múltiplo, são incomuns. É ainda mais raro como primeiro sinal de apresentação, ocorrendo em menos de 1% dos casos. A imunoglobulina associada mais comum a derrames pleurais malignos é o subtipo IgA (80%). Esta condição carrega um mau prognóstico. Nosso objetivo é descrever um caso refratário de mieloma múltiplo com doença extensa que apresentou recidiva extramedular com derrame pleural maligno, além de discutir a importância do diagnóstico diferencial