Hepatopatia associada a doença falciforme: uma revisão de literatura

Abstract

Sickle Cell Disease has a high prevalence in the population and may present with various vaso-occlusive crises throughout the life of the affected individual. These crises influence the morbidity and mortality of the disease as well as the patient's quality of life. The liver is one of the organs that can be affected by the vaso-occlusions characteristic of the disease, secondary to the sickling of the abnormal red blood cells in the hepatic sinusoids, which ultimately leads to sinusoidal obstruction and inflammation. Sickle Cell Hepatopathy is a broad term, and the differential diagnosis among the various conditions affecting the liver in sickle cell patients can be challenging, given the similar initial presentations and the wide spectrum of laboratory manifestations. The purpose of this article is to review the existing literature on liver disease in sickle cell disease, both in its acute and chronic forms. In doing so, it addresses relevant aspects of the pathophysiology, clinical presentation, and management of the main differential diagnoses, as these are rare and potentially severe manifestations that require prompt recognition and management to prevent unfavorable outcomes.