People whose hemoglobin does
not produce enough alpha protein have alpha thalassemia. It is
commonly found in Africa, the Middle East, India, Southeast
Asia, southern China, and occasionally the Mediterranean region.
There are many types of alpha thalassemia that range from
mild to severe in their effect on the body.
Silent Carrier State.
This condition generally causes no health problems because
the lack of alpha protein is so small that the hemoglobin
functions normally. It is called "silent carrier" because of
how difficult it is to detect. Silent carrier state is
"diagnosed" by deduction when an apparently normal
individual has a child with hemoglobin H disease or alpha
thalassemia trait.
Hemoglobin Constant Spring.
This is an unusual form of Silent Carrier state that is
caused by a mutation of the alpha globin. It is called
Constant Spring after the region of Jamaica in which it was
discovered. As in silent carrier state, an individual with
this condition usually experiences no related health
problems.
Alpha Thalassemia Trait
or Mild Alpha Thalassemia.
In this condition, the lack of alpha protein is somewhat
greater. Patients with this condition have smaller red blood
cells and a mild anemia, although many patients do not
experience symptoms. However, physicians often mistake mild
alpha thalassemia for iron deficiency anemia and prescribe
iron supplements that have no effect on the anemia.
Hemoglobin H Disease.
In this condition, the lack of alpha protein is great enough
to cause severe anemia and serious health problems such as
an enlarged spleen, bone deformities and fatigue. It is
named for the abnormal hemoglobin H (created by the
remaining beta globin) that destroys red blood cells.
Hemoglobin H-Constant Spring.
This condition is more severe than hemoglobin H disease.
Individuals with this condition tend to have a more severe
anemia and suffer more frequently from enlargement of the
spleen and viral infections.
Homozygous Constant
Spring.
This condition is a variation of hemoglobin H-Constant
Spring that occurs when two Constant Spring carriers pass
their genes on to their child (as opposed to hemoglobin H
Constant Spring, in which one parent is a Constant Spring
Carrier and the other a carrier of alpha thalassemia trait).
This condition is generally less severe than hemoglobin H
Constant Spring and more similar to hemoglobin H disease.
Hydrops Fetalis or
Alpha Thalassemia Major.
In this condition, there are no alpha genes in the
individual's DNA, which causes the gamma globins produced by
the fetus to form an abnormal hemoglobin called hemoglobin
Barts. Most individuals with this condition die before or
shortly after birth. In some extremely rare cases where the
condition is discovered before birth, in utero blood
transfusions have allowed the birth of children with hydrops
fetalis who then require lifelong blood transfusions and
medical care.