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Clinical signs include severe, rapid drop in hemoglobin leading to hypovolemic shock and death. Pediatric patients with sickle cell disease and beta thalassemia experience multiple splenic infarcts, resulting in splenic fibrosis and scarring. Over time, this leads to a small, auto infarcted spleen typically by the time patients reach adulthood.
Microcytic anaemia; Microcytosis is the presence of red cells that are smaller than normal. Normal adult red cell has a diameter of 7.2 µm. Microcytes are common seen in with hypochromia in iron-deficiency anaemia, thalassaemia trait, congenital sideroblastic anaemia and sometimes in anaemia of chronic diseases.
Related symptoms and signs include lethargy, decreased urine output, blood in the urine, kidney failure, low platelets, (which are needed for blood clotting), and destruction of red blood cells (microangiopathic hemolytic anemia).
Jaundice, also known as icterus, is a yellowish or greenish pigmentation of the skin and sclera due to high bilirubin levels. [3] [6] Jaundice in adults is typically a sign indicating the presence of underlying diseases involving abnormal heme metabolism, liver dysfunction, or biliary-tract obstruction. [7]
In terms of cause, almost any condition that involves ischemia can lead to renal papillary necrosis. A mnemonic for the causes of renal papillary necrosis is POSTCARDS: pyelonephritis, obstruction of the urogenital tract, sickle cell disease, tuberculosis, cirrhosis of the liver, analgesia/alcohol use disorder, renal vein thrombosis, diabetes mellitus, and systemic vasculitis. [3]
When Hemoglobin O-Arab co-inherits with Hemoglobin S, it produces a syndrome with similarities in severity to sickle cell anaemia, [1] having severe haemolytic anaemia and red cells denser than normal with occurrences of some cells as dense as if contract with sickle cell anaemia. A description with the presence of two variations of β-globin ...
Cold agglutinin disease (CAD) is a rare autoimmune disease characterized by the presence of high concentrations of circulating cold sensitive antibodies, usually IgM and autoantibodies that are also active at temperatures below 30 °C (86 °F), [1] directed against red blood cells, causing them to agglutinate and undergo lysis. [2]
282.5 Sickle-cell trait; 282.6 Sickle-cell anemia; 283 Acquired hemolytic anemias. 283.0 Autoimmune hemolytic anemias. Warm autoimmune hemolytic anemia; 283.1 Non-autoimmune hemolytic anemias; 283.2 Hemoglobinuria due to hemolysis from external causes Paroxysmal nocturnal hemoglobinuria; 284 Aplastic anemia. 284.0 Constitutional aplastic anemia