RESUMO
Inflammatory myofibroblastic tumors are rare tumors with an ALK (anaplastic lymphoma kinase) gene rearrangement in up to 65% of all cases. In our patient, the tumor was not primary resectable due to its extension. Under neoadjuvant treatment with the first generation ALK inhibitor crizotinib no tumor response was seen, but the following therapy with the next generation ALK inhibitor lorlatinib led to a rapid and deep response, enabling a complete tumor resection by partial cystectomy. Our case indicates that ALK positive inflammatory myofibroblastic tumors which do not respond to ALK inhibition with crizotinib can be successfully treated with newer agents.
Assuntos
Aminopiridinas/administração & dosagem , Quinase do Linfoma Anaplásico/genética , Crizotinibe/administração & dosagem , Fibronectinas/genética , Lactamas/administração & dosagem , Miofibroblastos/citologia , Proteínas de Fusão Oncogênica/genética , Pirazóis/administração & dosagem , Neoplasias da Bexiga Urinária/tratamento farmacológico , Neoplasias da Bexiga Urinária/genética , Adulto , Quinase do Linfoma Anaplásico/metabolismo , Antineoplásicos/administração & dosagem , Cistoscopia , Feminino , Fibronectinas/metabolismo , Fusão Gênica , Humanos , Inflamação , Neoplasias de Tecido Muscular/tratamento farmacológico , Neoplasias de Tecido Muscular/genética , Proteínas de Fusão Oncogênica/metabolismo , Inibidores de Proteínas Quinases/administração & dosagem , Resultado do TratamentoRESUMO
The storage of red blood cells (RBCs) leads to storage lesions, which have a negative impact on the clinical outcome after transfusion. A hallmark of storage lesions is echinocytosis. Albumin may reverse this shape transformation, which was the topic of this study. Echinocytosis was generated by incubation of blood for 48âh at room temperature or in RBC units stored 48 days at 5°C. Human serum albumin was diluted in phosphate-buffered saline. RBCs were fixed in 1% glutaraldehyde and examined by light and scanning electron microscopy. The degree of echinocytosis was quantified by calculating the morphological index. Incubation and storage of RBCs led to an echinocytic shape transformation, which was reversible upon incubation in albumin solutions. This process was time-, concentration- and hematocrit-dependent. Treating RBC units at the end of their shelf-life by adding 20% albumin or washing them in 0.2% albumin reversed all degrees of echinocytosis towards discocytosis. In conclusion, albumin has the capacity to reverse echinocytosis generated by RBC storage. This observation may improve the quality of RBC units stored for longer periods of time.