SYNDROME OF PAROXYSMAL-NOCTURNAL HEMOGLOBINURIA IN CHILDREN WITH ACQUIRED APLASTIC-ANEMIA

Syndrome of paroxysmal nocturnal hemoglobinuria (PNH) occurs frequentl y in aplastic anemia (AA). In our AA cases it was encounted in 10%. 6 cases are presented as illustration. PNH diagnosis was confirmed at po sitive Hem's test or flow cytofluorimetry basing on low expression of CD14 and CD16 on monocytes and granulocytes, respectively. PNH predict ors are: enhanced reticulocytosis, high activity of serum lactate dehy drogenase, elevated levels of indirect bilirubin, macrocytosis. In the treatment with ciclosporin A a transitory partial hematological respo nse was registered in 1 of 6 patients, metandrostenolon was uneffectiv e in 3 patients.