PYROPOIKILOCYTOSIS Meaning and
Definition
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Pyropoikilocytosis is a rare hereditary disorder affecting red blood cells, characterized by the presence of abnormal shaped cells, known as poikilocytes, that are susceptible to heat-induced hemolysis. The term is derived from pyro- meaning "fire," poikilo- meaning "varied" or "irregular," and cytosis meaning "condition of cells."
The abnormal red blood cells in pyropoikilocytosis exhibit various irregular shapes, including teardrop forms, elliptocytes, fragmented cells, and spherocytes. These distorted shapes impair the cells' ability to effectively transport oxygen throughout the body. Heat, particularly mild elevations in temperature, aggravates the fragility of these cells, leading to their premature destruction (hemolysis), resulting in anemia. Consequently, individuals with pyropoikilocytosis may experience symptoms such as fatigue, pale skin, shortness of breath, and jaundice.
Pyropoikilocytosis is an inherited condition that usually follows an autosomal recessive pattern, meaning that both parents must carry and pass on the abnormal gene for the disorder to develop in their child. The specific genetic mutations responsible for pyropoikilocytosis have not been extensively studied or identified.
Diagnosis of pyropoikilocytosis typically involves examining a blood smear under a microscope, where the characteristic abnormal red blood cell shapes can be observed. Genetic testing may also be performed to confirm the diagnosis and identify the specific mutations responsible.
Treatment for pyropoikilocytosis is primarily supportive and focuses on managing anemia and associated symptoms. This may include blood transfusions, iron supplementation, and folic acid supplementation to support red blood cell production. In severe cases, splenectomy (s
Etymology of PYROPOIKILOCYTOSIS
The word "pyropoikilocytosis" is composed of several Greek roots.
"Pyro-" comes from the Greek word "pyr", meaning fire. It can be a prefix indicating heat or fire-related concepts.
"Poi-", derived from the Greek root "poiēsis", means creation or production.
"Kilo-" stems from the Greek word "chilioi", meaning thousand.
"Cyt" comes from the Greek word "kytos", which means cell.
"-osis" is a suffix in medical terminology referring to a specific condition or abnormality.
Therefore, when these roots are combined, "pyropoikilocytosis" refers to the condition of having abnormally shaped red blood cells that are prone to being destroyed by heat or fire.
