[A cold agglutinin disease, difficult to distinguish from paroxysmal cold hemoglobinuria].
A 58-year-old male started passing dark brown colored urine in January 2002. An annual medical examination in the same month revealed a mild anemia and an increased serum LDH level. Because of the instability of the data of his peripheral red cell count and hemoglobin concentration as measuring with a blood cell auto-analyzer, and also the hemolytic findings in a test tube at room temperature (25 degrees C), he was referred to our hospital. Laboratory data revealed Hb 11.2 g/dl, reticulocytes 73.1% (233,000/microliter), indirect-bilirubin 2.8 mg/dl, LDH 757 U/l, and hemosiderinuria, suggesting some intravascular hemolysis. The cold agglutinin titer was > 1,024, direct and indirect Coombs tests were both positive, and the Donath-Landsteiner antibody (D-L antibody) was initially assessed as false positive because of a high titer of cold agglutinin. He was finally diagnosed as having a cold agglutinin disease (CAD) with anti-I autoantibody. Serologic tests for syphilis and mycoplasma, and various virus tests were all negatives. After avoiding cold exposure, his symptoms, including hemoglobinuria, disappeared. However, during 9 months follow-up, he still showed a high titer of cold agglutinin. This case suggests that CAD should be considered when peripheral blood cell count data are unstable as assessed by a blood cell auto-analyzer.
Yokoyama
Tomohisa
T
First Department of Internal Medicine, Tokyo Medical University.
Miyazawa
Keisuke
K
Sunaga
Kazuyo
K
Suzuki
Shigeru
S
Tanokura
Chizuru
C
Higakubo
Tosiaki
T
Nisizato
Yosinori
Y
Fukutake
Katsuyuki
K
Ohyashiki
Kazuma
K
D000373:Agglutinins / Q000097:blood; D000744:Anemia, Hemolytic, Autoimmune / Q000097:blood / Q000175:diagnosis*; D001323:Autoantibodies / Q000097:blood; D015415:Biomarkers / Q000097:blood; D001772:Blood Cell Count; D003080:Cold Temperature; D003937:Diagnosis, Differential; D005500:Follow-Up Studies; D006457:Hemoglobinuria, Paroxysmal; D006801:Humans; D007770:L-Lactate Dehydrogenase / Q000097:blood; D008297:Male; D008875:Middle Aged
D000373:Agglutinins; D001323:Autoantibodies; D015415:Biomarkers; C067477:Donath-Landsteiner antibody; D007770:L-Lactate Dehydrogenase