Paroxysmal Nocturnal Hemoglobinuria

PNH

Thromboses may occur in PNH at unusual sites In Paroxysmal Nocturnal Hemoglobinuria (PNH) there is a clone of abnormal hemopoietic stem cells in which there is a deficiency of molecules normally anchored to the membrane by phosphatidyl inositol linkages. The gene responsible for PNH is carried on X chromosome and both males and females may be affected. The red cell membranes are […]






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Acanthocytosis

Acanthocytes (spur cells) are spiculated red cells with a few projections of varying size and surface distribution. Acanthocytosis may be inherited (autosomal recessive) in association with retinitis pigmentosa, diffuse neurological deficits, and aß-lipoproteinemia.  Acanthocytes are also seen in renal failure, liver cirrhosis, microangiopathic hemolytic anemia, and as an artifact in blood stored in EDTA. Acanthocytes should be distinguished from Echinocytes […]






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Stomatocytosis

Stomatocytes (RBCs with slit-like central pallor) Stomatocytosis is a rare condition of RBCs in which a mouthlike or slitlike pattern replaces the normal central zone of pallor. These cells are associated with congenital and acquired hemolytic anemia. The symptoms result from the anemia. The rare congenital stomatocytosis, which shows autosomal dominant inheritance, causes a severe hemolytic anemia presenting very early […]






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Elliptocytosis

Hereditary Elliptocytosis (also known as ovalocytosis) is a disorder of the red cell membrane inherited usually in an autosomal dominant pattern. In this condition, the majority of cells have an elliptical shape. The osmotic fragility is normal. Most affected individuals are asymptomatic, though mild anemia, jaundice, splenomegaly and leg ulcers can occur. The diagnosis of hereditary elliptocytosis is usually made by […]






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Spherocytosis

Hereditary Spherocytosis (HS) is a disorder of the red cell membrane inherited usually in an autosomal dominant pattern. In this condition, the red cells are more rigid and fragile than normal. They are spherocytic in shape appearing small and deeply stained on blood smears, and have osmotic fragility. A normal red blood cell can live for up to 120 days. […]






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