Novel Insights into the Pathophysiology and Treatment of Sickle Cell Disease

被引：4

作者：

Araujo, Aderson da Silva ^{[1
]}

Pinto, Ana Cristina Silva ^{[2
]}

Lobo, Clarisse Lopes de Castro ^{[3
]}

Figueiredo, Maria Stella ^{[4
]}

Gualandro, Sandra Fatima Menosi ^{[5
]}

Saad, Sara Teresinha Olalla ^{[6
]}

Cancado, Rodolfo Delfini ^{[7
]}

机构：

[1] Fdn Hematol & Hemoterapia Pernambuco, Dept Hematol, Recife, Brazil

[2] Univ Sao Paulo, Fac Med Ribeirao Preto, Dept Med Imaging Hematol & Oncol, Hemoctr Ribeirao Preto,Hosp Clin, Ribeirao Preto, Brazil

[3] Inst Estadual Hematol Arthur de Siqueira Cavalcan, Dept Clin Res, Rio De Janeiro, Brazil

[4] Univ Fed Sao Paulo UNIFESP, Dept Clin & Expt Oncol, Escola Paulista Med, Sao Paulo, Brazil

[5] Univ Sao Paulo, Dept Hematol, Hosp Clin, Fac Med, Sao Paulo, Brazil

[6] Univ Estadual Campinas, Dept Internal Med, Fac Ciencias Med, Campinas, Brazil

[7] Fac Ciencias Med Santa Casa Sao Paulo, Dept Hematol & Oncol, Sao Paulo, Brazil

来源：

HEMOGLOBIN | 2023年 / 47卷 / 02期

关键词：

Sickle cell disease; pathophysiology; treatment; perspective; GARDOS CHANNEL BLOCKER; L-GLUTAMINE; SENICAPOC; CRIZANLIZUMAB; HYDROXYUREA; EXPRESSION; ADENOSINE; HEMOLYSIS; THERAPY; ANEMIA;

D O I：

10.1080/03630269.2023.2211771

中图分类号：

Q5 [生物化学]; Q7 [分子生物学];

学科分类号：

071010 ; 081704 ;

摘要：

The polymerization of hemoglobin under deoxygenation is the main pathophysiological event in sickle cell diseases, described more than 70 years ago. The last two decades have seen a major increase in knowledge about the cascade of events that follow the polymerization of hemoglobin and the ensuing sickling of red blood cells. Several distinctive therapeutic targets have been discovered as a result, and a few drugs with innovative mechanisms of action are already on the market, while several others are the focus of ongoing trials. The aim of this narrative review is to describe some of the more recent data in the SCD literature regarding pathophysiology and novel treatments.

引用

页码：71 / 79

页数：9

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