Hipertensão arterial pulmonar associada à anemia falciforme

Abstract

Pulmonary hypertension is a common complication of sickle cell anemia. Despite the fact that the elevations in pulmonary artery pressures are slight, morbidity and mortality are high. In adult sickle cell anemia patients, pulmonary hypertension is emerging as a major risk factor for death. The pathogenesis of sickle cell anemia-related pulmonary hypertension is multifactorial, including hemolysis, impaired nitric oxide bioavailability, chronic hypoxemia, thromboembolism, chronic liver disease and asplenia. In the majority of patients, pulmonary arterial hypertension is the main cause of elevated pulmonary artery pressures. However, pulmonary venous hypertension also plays a role in a subgroup of patients. Specific data on the effects of treatment modalities for pulmonary hypertension in patients with sickle cell anemia are scarce. It is likely that all patients would benefit from maximization of sickle cell anemia therapy, and that patients with the severe form of the disease would benefit from treatment with selective pulmonary vasodilators and antiproliferative agents. Large trials evaluating the effects of treatment for pulmonary hypertension in the sickle cell anemia population are underway.

Aspectos epidemiológicos e sociais da doença falciforme

“…A hipertensão arterial pulmonar é também uma complicação comum em pacientes com anemia falciforme; a morbimortalidade é alta e um fator de risco muito importante. 15 Essas comorbidades foram as mais referidas pelos pacientes do presente estudo, sendo a cardiopatia encontrada em quase metade deles e a hipertensão arterial em um quinto.…”

Sickle Cell Disease and Venous Thromboembolism

“…Hemolytic anemia through hemolysis and release of hemoglobin into plasma which consumes NO and release arginase into plasma leads to resistance to NO-dependent vasodilatory effects. This is an important process in the development of PHT and shortened life expectancy in patients with SCD 25,28. Bunn et al29 reviewed the literature related to the influence of NO depletion by plasma hemoglobin in the microcirculation on the pathogenesis of PHT in SCD patients.…”

Musculoskeletal pain, profile and quality of life of individuals with sickle cell disease

“…The HbS presents with different chemical characteristics and its functions are maintained under normal oxygenation. However, in situations of absence or reduced oxygen tension, the HbS undergoes polymerization changing its morphology and transforming into a sickle shape (sickling) resulting in ischemia, inflammation, disorders, pain and also chronic hemolysis that generates irreversible damage to various tissues and organs 2,[4][5][6] . Pain crises in this patient population are frequent and factors contributing to this process are: cold exposure, intense physical effort, hypoxia, dehydration, infections and general trauma 2,6 .…”