Asunto(s)
Antineoplásicos Alquilantes/uso terapéutico , Neoplasias del Sistema Nervioso Central/tratamiento farmacológico , Linfoma de Células T/tratamiento farmacológico , Temozolomida/uso terapéutico , Anciano , Encéfalo/efectos de los fármacos , Encéfalo/patología , Neoplasias del Sistema Nervioso Central/patología , Neoplasias del Sistema Nervioso Central/secundario , Femenino , Humanos , Linfoma de Células T/patologíaRESUMEN
Immunoglobulin light chain amyloidosis (AL) is a plasma cell disorder characterized by accumulation of misfolded proteins, which can induce organ damage. Venetoclax is active in multiple myeloma patients, in particular those with t(11;14) translocation. t(11;14) translocation is the most common cytogenetic abnormality in AL patients; venetoclax may thus be a useful additional treatment option for this disease. However, a recent trial in multiple myeloma patients (BELLINI) reported increased mortality associated with venetoclax versus placebo in combination with bortezomib and dexamethasone. In this report, we describe an AL patient who had suffered from recurrent infection during previous treatment, but who responded to and tolerated well single-agent venetoclax for more than 1 year. The present report indicates that venetoclax monotherapy may be active and safe for refractory AL amyloidosis.
Asunto(s)
Compuestos Bicíclicos Heterocíclicos con Puentes/administración & dosificación , Fragilidad , Amiloidosis de Cadenas Ligeras de las Inmunoglobulinas/tratamiento farmacológico , Sulfonamidas/administración & dosificación , Adulto , Compuestos Bicíclicos Heterocíclicos con Puentes/efectos adversos , Cromosomas Humanos Par 11/genética , Dexametasona/administración & dosificación , Dexametasona/efectos adversos , Quimioterapia Combinada/efectos adversos , Humanos , Amiloidosis de Cadenas Ligeras de las Inmunoglobulinas/genética , Masculino , Seguridad , Sulfonamidas/efectos adversos , Translocación Genética , Resultado del TratamientoRESUMEN
Disseminated intravascular coagulation (DIC) is characterized by systemic activation of coagulation pathway which can lead to thrombosis and bleeding. Chronic DIC associated with aortic aneurysm or dissection commonly presents with bleeding symptoms with variable severity due to enhanced fibrinolysis. Patients with unexplained bleeding diathesis with DIC should include aortic disease as one of the differential diagnoses. This paper reports two patients with severe hemorrhagic complications due to aortic disease. Long-term prophylactic treatment is recommended if the underlying aortic disease cannot be surgically managed. Tranexamic acid is a potentially safe option to relieve bleeding tendency.