Table of Contents
What is pyropoikilocytosis?
Hereditary pyropoikilocytosis (HPP) is a disease emanating from a defect in spectrin, which is the major peripheral protein of the red blood cell (RBC) membrane. This blood disorder is characterized by an RBC morphology similar to that seen in patients suffering from extensive burns—hence the term pyropoikilocytes.
How common is hereditary Pyropoikilocytosis?
Hereditary elliptocytosis (HE) is characterized by the presence of elliptical or oval erythrocytes on the blood films of affected individuals. The worldwide incidence of HE is estimated to be between one in 2,000 and one in 4,000 individuals.
What is Eliptocitosis?
Elliptocytosis is a hereditary disorder of the red blood cells (RBCs). In this condition, the RBCs assume an elliptical shape, rather than the typical round shape. Blood is comprised of red blood cells, platelets, and various white blood cells. Elliptocytosis is a hereditary disorder of the red blood cells (RBCs).
Is sickle cell a hemolytic anemia?
The term for destruction of red blood cells is “hemolysis.” There are two types of hemolytic anemia: Intrinsic — the destruction of the red blood cells due to a defect within the red blood cells themselves. Intrinsic hemolytic anemias are often inherited, such as sickle cell anemia and thalassemia.
What is an Elliptocyte?
Elliptocytes are elongated oval-shaped red blood cells. Very rare elliptocytes may be seen in normal blood smears. Elliptocytes may be increased in iron deficiency anemia (where they are sometimes referred to as “pencil cells”) and marrow infiltrative processes (with teardrop cells).
Why is sheep blood used in blood agar?
Sheep blood has been preffered source in the Blood agar due to the fact that sheep RBCs are most sensitive to the the hemolytic toxins released by bacterial cells thus causing hemolytic zones around the colonies over the period of time.
How is alpha different from hemolysis?
The main difference between alpha and beta hemolysis is that alpha hemolysis is involved in partial hemolysis associated with the reduction of hemoglobin in red blood cells whereas beta hemolysis is involved in the complete hemolysis of red blood cells that surround the colony.
What is anisocytosis and Anisokaryosis?
Abnormal Cell and Nuclear Size This is described as anisocytosis and anisokaryosis. The former refers to altered cell size whereas the later refers to altered nuclear size.
What is the history of pyropoikilocytosis?
Hereditary pyropoikilocytosis was originally described by Zarkowsky et al. (1975) as a distinct hemolytic anemia characterized by microspherocytosis, poikilocytosis, and an unusual thermal sensitivity of red cells.
What is hereditary pyropoikilocytosis (HPP)?
Hereditary pyropoikilocytosis (HPP) is an autosomal recessive form of hemolytic anemia characterized by an abnormal sensitivity of red blood cells to heat and erythrocyte morphology similar to that seen in thermal burns or from prolonged exposure of a healthy patient’s blood sample to high ambient temperatures.
How is hereditary pyropoikilocytosis detected by the eosin-5-maleimide (EMA)-binding test?
Detection of hereditary pyropoikilocytosis by the eosin-5-maleimide (EMA)-binding test is attributable to a marked reduction in EMA-reactive transmembrane proteins. King MJ, Jepson MA, Guest A, Mushens RInt J Lab Hematol2011 Apr;33(2):205-11.
What are the treatment options for pyropoikilocytosis hereditary?
Splenectomy is a possible treatment ^ “Pyropoikilocytosis hereditary | Genetic and Rare Diseases Information Center (GARD) – an NCATS Program”. rarediseases.info.nih.gov. Retrieved 17 July 2019. ^ Zarkowsky HS, Mohandas N, Speaker CB, Shohet SB (1975).