A blood diso rder caused by a lack of vitamin B12. Patients
who have this disorder do not produce the substance in the stomach that allows the
body to absorb vitamin B12. This substance is called intrinsic factor (IF).
Addison anemia, better known today as pernicious anemia (PA), is characterized
by the presence in the blood of large, immature, nucleated cells (megaloblasts)
that are forerunners of red blood cells. (Red blood cells, when mature, have no
nucleus). It is thus a type of megaloblastic anemia.
Pernicious anemia (PA) was first described (although not by that name) in 1855
by the English physician Thomas Addison. He called it an invariably fatal "idiopathic
anemia." The "idiopathic" was a frank admission that the cause of this illness was
wholly unknown. The name "pernicious anemia" was coined in 1872 by the German physician
Anton Biermer whose description of the disease was superior to that of Addison.
The studies of George H. Whipple on the effects of feeding liver in anemia followed
by those of George R. Minot and Wm. P. Murphy on the effects of feeding liver specifically
in pernicious anemia (PA) led to the cure of PA and to their receiving the Nobel
Prize in 1934.
Nowadays PA is an unpernicious anemia. It is simply treated with vitamin B12.
The vitamin B12 has to be administered by injection (parenterally) because people
with PA do not have IF (or an effective form of IF) and so cannot absorb vitamin
B12 taken by mouth.
There is some evidence that PA may be genetic although its mode of inheritance
is poorly documented. There is a congenital form of PA due to defect of IF that
is clearly inherited as an autosomal recessive trait with the affected child having
received two copies of the gene, one from each parent. The IF gene itself has been
localized to human chromosome 11.
The word "pernicious" means highly injurious, destructive, or deadly. "Pernicious"
comes from the Latin root "nex" meaning "violent death." Pernicious anemia was once
quite deadly. Today it fortunately is not.
PA has also been called addisonian anemia and Biermer's anemia.
Common Misspellings: addison anaema, addison enemia, addison anaemia, addison
aneamia
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