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 patients blood sample to high ambient temperatures. Patients with HPP tend to experience severe hemolysis and anemia in infancy that gradually improves, evolving toward typical elliptocytosis later in life. However, the hemolysis can lead to rapid sequestration and destruction of red cells. Splenectomy is curative when this occurs. HPP has been associated with a defect of the erythrocyte membrane protein spectrin and with spectrin deficiency. It was characterized in 1975. It is considered a severe form of hereditary elliptocytosis. Causes
Mutations of the alphaspectrin gene causes this disease. HPP can be considered as a subset of hereditary elliptocytosis. Diagnosis
Genetic testing for the presence of mutations in protein molecules is considered to be a confirmatory testing technique. It is important to know the risks regarding the transmission and dangers of HPP. Treatment
Splenectomy is a possible treatment

See also
Erythrocyte
Poikilocytosis
List of hematologic conditions

References


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A varus deformity is an excessive inward angulation (medial angulation, that is, towards the bodys midline) of the distal segment of a bone or joint. The opposite of varus is called valgus. EX: Varus deformity results in a decreased Q angle of the knee joint. The terms varus and valgus always refer to the direction that the distal segment of the joint points. For example, in a valgus deformity of the knee, the distal part of the leg below the knee is deviated outward, in relation to the femur, resulting in a knock-kneed appearance. Conversely, a varus deformity at the knee results in a bowlegged  with the distal part of the leg deviated inward, in relation to the femur. However, in relation to the mid-line of the body, the knee joint is deviated towards the mid-line. EX: Valgus deformity results in an increased Q angle of the knee joint. Terminology
The terminology is made confusing by the etymology of these words. The terms varus and valgus are both Latin, but confusingly, their Latin meanings conflict with their current usage. In current usage, as noted above, a varus deformity of the knee describes bowed legs, but in the original Latin, varus meant "knock-kneed."