Vaso-occlusive crisis in sickle cell disease: a vicious cycle of secondary events.
J Transl Med
; 19(1): 397, 2021 09 20.
Article
en En
| MEDLINE
| ID: mdl-34544432
ABSTRACT
Painful vaso-occlusive crisis (VOC) remains the most common reason for presenting to the Emergency Department and hospitalization in patients with sickle cell disease (SCD). Although two new agents have been approved by the Food and Drug Administration for treating SCD, they both target to reduce the frequency of VOC. Results from studies investigating various approaches to treat and shorten VOC have so far been generally disappointing. In this paper, we will summarize the complex pathophysiology and downstream events of VOC and discuss the likely reasons for the disappointing results using monotherapy. We will put forward the rationale for exploring some of the currently available agents to either protect erythrocytes un-involved in the hemoglobin polymerization process from sickling induced by the secondary events, or a multipronged combination approach that targets the complex downstream pathways of VOC.
Palabras clave
Texto completo:
1
Bases de datos:
MEDLINE
Asunto principal:
Anemia de Células Falciformes
Límite:
Humans
Idioma:
En
Revista:
J Transl Med
Año:
2021
Tipo del documento:
Article
País de afiliación:
Estados Unidos