What are the types of alpha thalassemia?
Two types of alpha thalassemia can cause health problems. The more serious type called hemoglobin Bart hydrops fetalis syndrome or Hb Bart syndrome. The lighter form called HCB disease.
HB Bart syndrome is characterized by hydrops fetalis, a condition in which excessfluid accumulation in the body before birth. Additional signs and symptoms may include severe anemia, an enlarged liver and spleen (hepatosplenomegaly), heart defects and abnormalities of the urinary system or genitals. As a result of theseserious health problems, most babies with this condition are stillborn or die shortly after birth. Hb Bart syndrome may also lead to serious complications for women during pregnancy, including dangerously high blood pressure with swelling (preeclampsia), premature delivery,
and abnormal bleeding.
HBH disease causes mild to moderate anemia, hepatosplenomegaly, and yellowing of the eyes and skin (jaundice). Some affected individuals also have been changes such as overgrowth of the upper jaw and an unusually prominentforehead. The characteristics of BH disease usually
appears in
early childhood,and affected individuals usually live into adulthood.
How common is Alpha thalassemia?
Alpha-thalassemia is a fairly common blood disorder worldwide. Thousands of babies with Hb Bart syndrome and HBH disease are born each year, especiallyin Southeast Asia. Alpha thalassemia occurs often in people of Mediterranean countries, North Africa, Middle East, India and Central Asia.