Eleven cases with cold agglutinin disease in the Department of Internal Medicine,
Yonsei University College of Medicine were evaluated over 20- year period (January
1968 through Decemvir 1987). The results are as followings:
1. Of forty seven cases with autoimmune hemolytic anemia, eleven(23.4%) were cold
agglutinin disease. Mean age at the diagnosis was 44.7 years(17-73 years) with female
predominance. Five cases(45%) were idiopathic and the other six(55%) were secondary
type of the disease in which systemic lupus erythematosus was the most common(67%)
underlying disease.
2. The most frequent symptom was dizziness(64%), Dark urine was noticed in four
cases(36%), acrocyanosis in 1 (18%). Physical examination showed hepatomegaly in 2
and splenomegaly in one.
3. The cold agglutinin titers in theree cases(27%) were above 1.1,024. Majority of
cases, seven of eleven (64%), had rather low cold agglutinin titer ( < 1 : 128). The
direct antiglobulin test was positive in all instances ; in 88% (7/8) positive for anti-lgG,
75% (6/8) for anti-complement and 63% (5/8) for both IgG and complement. Indirect
antiglobulin test was positive in 5 casas (45%).
4. The mean hemoglobin level was 6.9 ± 2.6g/dl and the mean red cell indices were
increased (MCV 98.1 土 16.9 fL, MCH 37.2 ± 9.3 pg, MCHC 38.5 ± 9.6%) The mean
reticulocyte count was 4.3 ± 4.1%. The total bilirubin level was increased in 9 cases
and LDH level was increased in 10cases, with a mean value of 4.5 ± 7.1 mg/dl and 488
± 522 lU/L, respectively. Urine hemoglobin and hemosiderin was positive in 4(36%) and
2(18), respectively.
5. Of eight cases who performed peripheral blood examination, all the cases had
polychromasia and five (63%) spherocytosis and four (50%) red cell agglutination. All
the cases showed hypercellulality and erythroid hyperplasia on bone marrow
examination.
6. Eight (73%) cases were transfused without transfusion reaction. Steroid was
administered in seven. Among them five responded with clinical and laboratory
improvements, one died due to acute renal failure and one with chronic
myeloproliferative disease responded to busulfan.
Upon the above results, the diagnosis of cold agglutinin disease should be born in
mind in case of autoimmune hemolytic aniemia, especially in cases with systemic lupus
erythematosus. And it might be very important for successful treatment to know the
underlying discase, significance of thermal amplitude and agglutinin titer.

Keywords: Cold agglutinin disease, hemolytic anemia