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The aplastic anemia-paroxysmal nocturnal hemoglobinuria syndrome

✍ Scribed by Marcel E. Conrad; James C. Barton


Publisher
John Wiley and Sons
Year
1979
Tongue
English
Weight
445 KB
Volume
7
Category
Article
ISSN
0361-8609

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✦ Synopsis


Abstract

Occasionally it is difficult to differentiate paroxysmal nocturnal hemoglobinuria (PNH) from idiopathic aplastic anemia in patients who present with pancytopenia and an aplastic bone marrow. Patients with PNH may not have an abnormal acid hemolysis test, and patients with aplastic anemia may present with evidence of abnormal sucrose lysis, acid hemolysis, and antibody‐mediated complement hemolysis. Demonstration of a population of red blood cells which are highly susceptible to antibody‐mediated complement lysis makes a diagnosis of PNH probable. Donor red blood cell survival studies, which distinguish intracorpuscular from extracorpuscular hemolytic disorders, permit differentiation of the two disorders.


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Lactic acidosis secondary to severe anem
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A patient with paroxysmal nocturnal hemoglobinuria developed lactic acidosis associated with severe anemia. The lactic acidosis corrected after blood transfusion. In the absence of shock, sepsis, or other identifiable causes of lactic acidosis, the severe anemia (hemoglobin 1.2 g/dl) appeared to be