Osteonecrosis in Sickle-Cell Disease

Therapy with bone marrow mesenchymal stem cells in bone regeneration in children with osteonecrosis secondary to sickle cell disease

“…Sickle cell disease (SCD) is a hereditary disease with autosomal recessive inheritance, where the mutation of hemoglobin can generate complications related to both hemolysis and microvascular occlusion. Conversely, sickle cell anemia (AF) is a type of hemolytic anemia characterized by the presence of red blood cells with altered morphology—sickle-shaped appearance—which are removed from circulation and destroyed at a faster rate ( Daltro and Hernigou, 2014 ). Vascular occlusion caused by the morphological alteration of red blood cells is the main factor in this disease, often leading to ischemia of various organs and tissues ( Daltro and Hernigou, 2014 ).…”

“…Conversely, sickle cell anemia (AF) is a type of hemolytic anemia characterized by the presence of red blood cells with altered morphology—sickle-shaped appearance—which are removed from circulation and destroyed at a faster rate ( Daltro and Hernigou, 2014 ). Vascular occlusion caused by the morphological alteration of red blood cells is the main factor in this disease, often leading to ischemia of various organs and tissues ( Daltro and Hernigou, 2014 ). A consequence of this alteration is the loss of elasticity and deformability, leading to increased viscosity in the cytosol and greater adherence of erythrocytes to the endothelium, reducing their fluidity and favoring the formation of thrombi in micro and macrocirculation with consequent vaso-occlusive phenomena ( Galiza Neto and Pitombeira, 2003 ).…”

“…Considering that AF primarily affects children and young people, musculoskeletal complications generate a high social and financial burden, as they require permanent medical follow-up and prevent these individuals from having a normal productive life ( Cançado and Jesus, 2007 ). Most of the time, the clinical course of the disease is asymptomatic until the onset of limiting pain, followed by structural bone deformity and loss of joint function in the individual ( Daltro and Hernigou, 2014 ). Hence, early diagnosis is extremely important for safer, more effective, and less invasive treatment before the only treatment alternative is complete joint replacement ( Daltro et al, 2008 ).…”

Osteonecrose da cabeça femoral: Artigo de atualização

“…A ONCF tem como etiologia uma vasta série de patologias que levam ao comprometimento da circulação microvascular da cabeça femoral, 3 4 seja por interrupção mecânica vascular, como em fraturas da cabeça femoral, fraturas de colo (especialmente as fraturas de colo desviadas – Garden III ou IV –,cujas sínteses são mantidas 5 ), ou luxação da articulação coxo femoral, em que há lesão da artéria circunflexa femoral medial; 6 oclusão intravascular, como nas discrasias sanguíneas (anemia falciforme, provavelmente a condição hematológica com mais rápida evolução clínica, 7 8 hemofilia, que causa repetidas hemorragias intraósseas, doença de von Willebrand, mutação do fator V de Leiden, deficiência de proteínas C e S, e policitemia vera 9 ); compressão extravascular, por acúmulo de gordura na medula óssea, como com o uso de corticoesteroides, 10 11 em que pode acontecer uma substituição de células pluripotentes por células adiposas, alteração da homeostase óssea, e dano aos osteócitos; e abuso de álcool. 12 13 Quanto ao uso de corticosteroides e o abuso de álcool comprovou-se que dependem da dose, com aumento do risco com o uso de mais de 20mg por dia de corticoides, e aumento de risco de quase 18 vezes com a ingestão de mais de 1.000 mL por semana de álcool.…”

“…The etiology of ONFH comprises a vast series of pathologies that lead to impairment of the microvascular circulation of the femoral head, 3 4 either by mechanical vascular interruption, as in femoral head fractures, femoral neck fractures (especially displaced neck fractures – Garden III or IV – whose syntheses are maintained) 5 or dislocation of the hip joint, in which there is injury to the medial circumflex femoral artery; 6 intravascular occlusion, such as in blood dyscrasias (sickle cell anemia – probably the hematological condition with the fastest clinical evolution, 7 8 hemophilia – causing repeated intraosseous hemorrhages, Von Willebrand disease, factor V Leiden mutation, deficiency of proteins C and S, and polycythemia vera) 9 ; extravascular compression, due to accumulation of fat in the bone marrow, such as with the use of corticosteroids, 10 11 in which there may be a replacement of pluripotent cells by fat cells, alteration of bone homeostasis, and damage to osteocytes; and alcohol abuse. 12 13 As for the use of corticosteroids and alcohol abuse, both have been proven to be dose-dependent, with an increase in the risk with the use of more than 20 mg a day of corticosteroids and an almost 18-fold increase in the risk with an intake of more than 1,000 mL a week of alcohol.…”