2018 Volume 67 Issue 3 Pages 373-378
A 47-year-old male patient noticed a reddish brown coloration of his urine, and visited our hospital. The urinalysis revealed strongly positive occult blood and microscopically few red cells. In spite of a slightly elevated urinary myoglobin level, clinical manifestations and an increased activity of creatine kinase associated with myoglobinuria were not found. Presence of free hemoglobin in serum indicated hemoglobinuria. Increased activity of serum LD (especially of LD1), AST, and hyperbilirubinemia (indirect being dominant), a decreased haptoglobin level, and reticulocytosis indicated the presence of hemolytic anemia. However, the results of direct and indirect Coombs tests were negative. Thus, autoimmune hemolytic anemia (AIHA) was not confirmed. Paroxysmal nocturnal hemoglobinuria (PNH) that gave rise to hemoglobinuria was ruled out by absence of PNH cells shown by CD 55/59 analysis. His cold agglutinin titer was normal. He had no history of congenital hemolytic anemia. The fact that his hemolytic episode with intermittent hemoglobinuria appeared during the coldest season of the year, in addition to the low agglutinin titer and negativity for PNH cells, suggested paroxysmal cold for hemoglobinuria (PCH). The Donath–Landsteiner (DL) test was performed two weeks after the first visit. However, the results were negative. The hemolytic episode seemed to have been maximal a few weeks before the 1st visit, and it almost terminated by the 2nd visit (2 weeks later) when the DL test was carried out. Although, no conclusive diagnosis for PCH was achieved, PCH was the most likely diagnosis being considered on the basis of the hemolytic episode with intermittent hemoglobinuria in a relatively short period during the coldest season. Timely performance of the DL test would be mandatory for the diagnosis of PCH on hemolytic involvement with hemoglobinuria during the cold season.
