Premium
The aplastic anemia‐paroxysmal nocturnal hemoglobinuria syndrome
Author(s) -
Conrad Marcel E.,
Barton James C.
Publication year - 1979
Publication title -
american journal of hematology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 2.456
H-Index - 105
eISSN - 1096-8652
pISSN - 0361-8609
DOI - 10.1002/ajh.2830070108
Subject(s) - paroxysmal nocturnal hemoglobinuria , aplastic anemia , hemolysis , pancytopenia , hemoglobinuria , medicine , immunology , hemolytic anemia , bone marrow
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.