Hereditary Pyropoikilocytosis is a rare, inherited condition in which abnormal red blood cells become sensitive to heat. As a result of this increased sensitivity, they get destroyed resulting in haemolytic anaemia. Symptoms are:
• Severe haemolytic anemia
• Growth retardation
• Protruding forehead
• Extreme poikilocytosis
• Oval shaped red blood cells
HOW IS HEREDITARY PYROPOIKILOCYTOSIS DIAGNOSED?
Haematologists may order for a complete blood count and a peripheral blood smear for diagnosis of Hereditary Pyropoikilocytosis. The doctor may conduct a complete physical examination and also evaluate your symptoms. Thermal sensitivity and osmotic fragility tests may confirm diagnosis.
HOW IS HEREDITARY PYROPOIKILOCYTOSIS TREATED?
Blood transfusion of packed red blood cells is the first line of treatment for Hereditary Pyropoikilocytosis. Supportive care like intravenous fluids, oxygen and constant monitoring helps alleviate symptoms and aid speedy recovery. Surgeries and other treatment options vary with the individual.
DID YOU KNOW?
Hereditary Pyropoikilocytosis is usually caused when both parents have particular problems.