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7. Categories of Immune Disorders
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7.2 Autoimmune Disease:
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7.2.3 Organ-specific autoimmune disease
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Pernicious anemia (autoimmune gastritis)
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- A chronic progressive anemia of older adults
(occurring more frequently during the
fifth and later decades, rarely prior to 30 years of age), due to failure of absorption of
vitamin B12, usually resulting from a defect of the stomach accompanied by mucosal
atrophy and associated with lack of secretion of intrinsic factor; characterized by
numbness and tingling, weakness, and a sore smooth tongue, as well as dyspnea after
slight exertion, faintness, pallor of the skin and mucous membranes, anorexia,
diarrhea, loss of weight, and fever; laboratory studies usually reveal greatly decreased
red blood cell counts, low levels of hemoglobin, numerous characteristically oval
shaped macrocytic erythrocytes (color index greater than normal, but not truly
hyperchromic), and hypo- or achlorhydria, in association with a predominant number
of megaloblasts and relatively few normoblasts in the bone marrow; the leukocyte
count in peripheral blood may be less than normal, with relative lymphocytosis and
hypersegmented neutrophils; a low level of vitamin B12 is found in peripheral red
blood cells; administration of vitamin B12 results in a characteristic reticulocyte
response, relief from symptoms, and an increase in erythrocytes, provided that
pernicious anemia is not complicated by another disease; the condition is not actually
pernicious, as it was prior to the availability of therapy with vitamin B12.
At least
two autosomal recessive forms are known. In one there is a defect of intrinsic factor
and in the other a defective absorption of vitamin B12 from the intestine. Syn: Biermer
anemia, addisonian anemia, macrocytic achylic anemia, Biermer disease, malignant
anemia, Addison anemia, Addison-Biermer disease.



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