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1.
Eur J Haematol ; 106(5): 654-661, 2021 May.
Artigo em Inglês | MEDLINE | ID: mdl-33523540

RESUMO

INTRODUCTION: Hemophagocytic lymphohistiocytosis (HLH) is a cytokine storm syndrome associated with mortality rates of up to 88%. Standard therapy with high-dose glucocorticoids and etoposide used in adults is extrapolated from pediatric trials, with significant toxicity in older patients and those with poor performance status. The JAK1/2 inhibitor ruxolitinib has recently gained attention as a treatment option for HLH due to its broad cytokine-modulating abilities and safety profile. Herein we report our center's experience using ruxolitinib in the treatment of adult-onset secondary HLH. CASE SERIES: We report four patients with profound secondary HLH provoked by diverse triggers, including invasive pulmonary aspergillosis on background systemic lupus erythematosus, disseminated tuberculosis, and T-cell lymphoma treated with ruxolitinib as monotherapy or combination therapy in upfront and salvage settings. RESULTS: All four patients had rapid, sustained improvement in clinical status, inflammatory markers, and hematological cell counts followed by durable remission. Three patients developed manageable infectious complications postruxolitinib. CONCLUSIONS: This series demonstrates the effective use of JAK inhibition with ruxolitinib to control pathological immune activation in critically ill patients with secondary HLH and otherwise limited therapeutic options. JAK inhibition is also an area of urgent investigation for the treatment of cytokine storm associated with COVID-19.


Assuntos
Janus Quinases/antagonistas & inibidores , Linfo-Histiocitose Hemofagocítica/tratamento farmacológico , Inibidores de Proteínas Quinases/uso terapêutico , Pirazóis/uso terapêutico , Adulto , COVID-19/complicações , Terapia Combinada , Síndrome da Liberação de Citocina/tratamento farmacológico , Síndrome da Liberação de Citocina/etiologia , Citocinas/sangue , Feminino , Humanos , Aspergilose Pulmonar Invasiva/complicações , Lúpus Eritematoso Sistêmico/complicações , Linfo-Histiocitose Hemofagocítica/etiologia , Linfoma de Células T/complicações , Masculino , Pessoa de Meia-Idade , Nitrilas , Pirimidinas , SARS-CoV-2 , Terapia de Salvação , Tuberculose/complicações , Adulto Jovem
2.
J Pain Palliat Care Pharmacother ; 35(1): 48-51, 2021 Mar.
Artigo em Inglês | MEDLINE | ID: mdl-33600270

RESUMO

There are currently limited published case reports and clinical studies looking at octreotide as a potential therapeutic agent for treating surgery- and malignancy-related chylothorax in adult patients. Few case reports have shown that low-dose subcutaneous octreotide can be used to treat malignant chylothorax. We report the case of a 57-year-old high-grade follicular lymphoma patient with malignant chylothorax which responded rapidly and was successfully treated with octreotide. Significant improvements were noted in her dyspnea, abdominal distention and pain, and chylous output. This case also highlights the importance of understanding the pharmacotherapeutic effects of octreotide when managing malignant chylothorax as it may help to benefit patients by improving symptoms, quality of life, and length of hospital stay. Further prospective studies are warranted to further evaluate the role of octreotide in the management of malignant chylothorax.


Assuntos
Quilotórax , Octreotida , Adulto , Quilotórax/tratamento farmacológico , Quilotórax/etiologia , Feminino , Humanos , Tempo de Internação , Pessoa de Meia-Idade , Octreotida/uso terapêutico , Qualidade de Vida
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