¿Qué son los acantocitos?
Los acantocitos son hematíes cuya forma se parece a la de las hojas de acanto. Esta planta mediterránea se caracteriza por el tamaño importante de sus hojas. Dotados de múltiples espículas y proyecciones en superficie estos glóbulos rojos constituyen una anomalía que se asocia, generalmente, a unos lípidos que tienen un metabolismo anormal.
¿Cuál es la morfología de los acantocitos?
Los acantocitos son glóbulos rojos con una morfología anómala. También se conocen como células espinosas, espiculadas o en espuela.
¿Cómo se diagnostica la acantocitosis?
¿Cómo se diagnostica la acantocitosis? La acantocitosis se refiere a una presencia anormal de acantocitos en la sangre. Estos glóbulos rojos deformes se pueden ver en un frotis de sangre periférica. Esto implica colocar una muestra de sangre en un portaobjetos de vidrio, teñirla y mirarla con un microscopio.
¿Cuál es la anormalidad de los acantocitos?
La anormalidad de los acantocitos en este síndrome se caracteriza por la presencia de gran cantidad de esfingolípidos en hoja externa de la bicapa de la membrana celular causando un aumento de la superficie que provoca la deformación. El síndrome de Bassen-Kornzweig siempre va acompañado de acantocitosis.
What causes acanthocytosis?
Acanthocytes can be caused by (1) altered distribution or proportions of membrane lipids or by (2) membrane protein or membrane skeleton abnormalities. In membrane lipid abnormalities, previously normal red cell precursors often acquire the acanthocytic morphology from the plasma.
What causes spur cells?
Historically, spur cell anemia has been associated with advanced alcoholic liver cirrhosis, but it is also seen in other types of severe liver disease. Acanthocytosis has also been associated with inherited neurologic disorders, aptly named neuroacanthocytosis syndromes.20-Jun-2021
Why are acanthocytes seen in Abetalipoproteinemia?
Acanthocytes are abnormally spiked RBCs due to the defective phospholipid cell membrane. They are also seen in liver dysfunction. Because of their inability to form rouleaux, erythrocyte sedimentation rates could be very low.11-Aug-2021
What does Anisocytosis mean in a blood test?
Anisocytosis is the medical term for having red blood cells (RBCs) that are unequal in size. Normally, a person's RBCs should all be roughly the same size. Anisocytosis is usually caused by another medical condition called anemia.
How is spur cell anemia diagnosed?
The peripheral blood smear (Figure 1) showed numerous irregularly shaped erythrocytes with spinous projections (ie, acanthocytes, or “spur cells”) and increased reticulocytes, confirming the diagnosis of spur-cell anemia.01-Nov-2020
What happens in individuals with spur cell anemia?
Spur cell anemia is an acquired hemolytic anemia associated with liver cirrhosis and is characterized by the presence of increased large red blood cells, which are covered with spike-like projections that vary in width, length, and distribution.29-May-2020
What do acanthocytes indicate?
Acanthocytes have an abnormal amount of these fats, or lipids, in odd proportions. That means the inner and outer surface areas of the blood cells are imbalanced. This causes them to harden, pucker, and form spikes. Severe liver disease is a common cause of acanthocytosis.28-May-2021
How are acanthocytes different from echinocytes?
Acanthocytes are irregularly spiculated cells (spicules are irregular in size, shape and distribution around the RBC membrane), whereas echinocytes are regularly spiculated cells.
What is the significance of Schistocytes?
The presence of schistocytes (fragmented red blood cells) on the peripheral blood smear suggests red blood cell injury from damaged endothelium and is a characteristic feature of microangiopathic hemolytic anemia.
What is a normal anisocytosis level?
Anisocytosis is reported as “slight” to 4+ (“four plus”) and gives the same information as the RDW parameter (red blood cell distribution width): the larger the size variation in the red blood cells, the higher the anisocytosis and RDW results will be.
Can Covid cause anisocytosis?
Anisocytosis is Associated With Short-Term Mortality in COVID-19 and May Reflect Proinflammatory Signature in Uninfected Ambulatory Adults. and On Behalf Of Inaction Study Group.02-Oct-2020
What anemia causes anisocytosis?
Anisocytosis may be present in several types of anemia, including the following. Iron deficiency anemia. Red blood cells may be abnormally small and pale in this type of anemia. Iron deficiency is most often caused by blood loss.24-Apr-2021
Acerca de los acantocitos: de dónde vienen y dónde se encuentran
Se cree que los acantocitos son el resultado de cambios en las proteínas y los lípidos en las superficies de los glóbulos rojos. Exactamente cómo y por qué se forman los picos no se entiende completamente.
Acantocitos vs equinocitos
Un acantocito es similar a otro glóbulo rojo anormal llamado equinocito. Los equinocitos también tienen picos en la superficie celular, aunque son más pequeños, de forma regular y espaciados de manera más uniforme en la superficie celular.
Lo que aprendiste
Los acantocitos son glóbulos rojos anormales que tienen picos irregulares en la superficie celular. Están asociados con afecciones hereditarias raras, así como con afecciones adquiridas más comunes.
Usage
Spur cells may refer synonymously to acanthocytes, or may refer in some sources to a specific subset of 'extreme acanthocytes' that have undergone splenic modification whereby additional cell membrane loss has blunted the spicules and the cells have become spherocytic ('spheroacanthocyte'), as seen in some patients with severe liver disease.
Pathophysiology
Acanthocytes arise from either alterations in membrane lipids or structural proteins. Alterations in membrane lipids are seen in abetalipoproteinemia and liver dysfunction. Alteration in membrane structural proteins are seen in neuroacanthocytosis and McLeod syndrome.
Differential diagnoses
The diagnosis of acanthocytosis should be differentiated from: acute or chronic anemia, hepatitis A, B, and C, hepatorenal syndrome, hypopituitarism, malabsorption syndromes, and malnutrition.
What is the treatment for acanthocytosis?
Treatment of disorders with acanthocytosis depends on the underlying condition. Medical care in abetalipoproteinemia includes dietary restriction of long-chain fatty acids, with judicious supplementation with medium-chain triglycerides. Supplementation with lipid-soluble vitamins A, D, E, and K is necessary in large doses.
What is an acanthocyte?
Acanthocytes (from the Greek word acantha, which means thorn), or spur cells, are spiculated red cells with a few projections of varying size and surface distribution (see the images below). Studies in acanthocytosis workup include a complete blood count (CBC) and a peripheral blood smear. Management of acanthocytosis depends on ...
What is the McLeod red cell phenotype?
Chronic granulomatous disease (CGD) associated with McLeod red cell phenotype. Acanthocytes should be distinguished from echinocytes (from the Greek word echinos, which means urchin). Echinocytes, or burr cells, appear with multiple small projections that are uniformly distributed on the red cell surface (see the image below).
What causes acanthocytes?
Acanthocytes can be caused by (1) altered distribution or proportions of membrane lipids or by (2) membrane protein or membrane skeleton abnormalities. In membrane lipid abnormalities, previously normal red cell precursors often acquire the acanthocytic morphology from the plasma.
What is the KX antigen?
Individuals with the McLeod blood group or McLeod syndrome lack the Kx antigen, a membrane precursor of the Kell antigen that leads to acanthocytic red cell morphology. The Kell antigen is located on a 93-kD glycoprotein and is associated with the underlying membrane skeleton.
How long does it take for a neonatal hepatitis to resolve?
In neonatal hepatitis, the process resolves in 65% of individuals within weeks to months.
Can acanthocytosis cause pale stools?
Patients with acanthocytosis may have a history of chronic diarrhea with pale, foul-smelling, and bulky stools; loss of appetite and vomiting; and slow weight gain and decreased growth, possibly with a bleeding tendency. Patients may report symptoms of ataxia, tremors, visual abnormalities, jaundice, abdominal pain, pallor, dark urine, and recurrent infections. Adolescents and adults may report dyskinesias, specifically orolingual, and cognitive deterioration.
Caracteristicas
Em geral, são pequenas células com espículas esparsas, de comprimento e largura variáveis, e distribuídas irregularmente na superfície da membrana. As proteínas que compõem a membrana dos acantócitos são normais, enquanto o conteúdo lipídico não.
Patologias relacionadas à presença de acantócitos
Diversas doenças apresentam a presença dessas hemácias anormais, entretanto, é muito importante diferenciar algumas dessas patologias anêmicas em que o número de acantócitos varia consideravelmente, coexistindo com hemácias normais.
Abetalipoprotoinemia congênita ou síndrome de Bassen-Kornzweig
Esta é uma síndrome autossômica recessiva hereditária que envolve a ausência congênita de aloproteína-β do plasma, uma proteína que está envolvida no metabolismo lipídico.
Acantocitose hereditária
A acantocitose no adulto está frequentemente associada a doença hepatocelular alcoólica grave (cirrose alcoólica) ou anemia hemolítica com acantócitos.
Neuroacantocitose
A acantocitose costuma ocorrer ocasionalmente em associação com uma ampla gama de doenças neurológicas: entre as quais estão atrofia muscular do tipo Charcot-Marie-Tooth, coreia-acantose, síndrome de MacLeod, entre outras, que estão agrupadas sob o nome de neuroacantocitose.
Síndrome de McLeod
É também uma doença cromossômica, ligada ao cromossomo X, na qual o sistema neuromuscular, nervoso e hematológico está comprometido. Hematologicamente, é caracterizado pela ausência de expressão do antígeno Kx do eritrócito, fraca expressão dos antígenos Kell e sobrevivência dos eritrócitos (acantócitos).
Outros distúrbios com presença de acantócitos
Os acantócitos podem ser vistos em pequeno número, em pessoas com problemas de desnutrição (anemia), com hipotireoidismo, após a remoção do baço (esplenectomia) e em pessoas com HIV, talvez devido a alguma deficiência nutricional.