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KMID : 0360919690120020150
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Journal of the Korean Medical Association
1969 Volume.12 No. 2 p.150 ~ p.156
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COLD AGGLUTININ DISEASE-REPORT OF A CASE
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鄭圭源/Chung, Kyu Won
羅秀燮/全鍾暉/李鍾武/Lah Soo Sup/Chun, chong Hwee/Lee, Chong Moo
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Abstract
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History: C. O. 0. 36 Male, Korean.
Patient was first admitted to Kyungbook National University Hospital, Departmant of Medicine on Dec. 8, 1966 because of sudden onset of pyrexia, chills, headache, dizziness and general weakness of about one week´s standing.
Approximately one week after onset of above symptoms, patient was noticed to be yellow by his family. Patient had been a rather heavy drinker over the past ten years.
On admission, patient was found to be moderately anemic, general condition was fair, vital signs were normal, icteric skin, faint precordial systolic murmur was heard, lungs were clear, slight hepatosplenomegaly was noted and neurological examinations were negative. Patient was. admitted with provisional diagnosis of hemolytic anemia.
After admission, transfusion was given which caused numerous episodes of "side-reaction" consisting of fever, chills and headache.
Approximately one month after onset of symptoms patient was transferred to Department of Internal Medicine, Catholic Medical Center, Seoul, Korea for further care and evaluation.
Laboratory findings:
C.B.C.; on admission, hemoglobin was 5.0 gm/ dl., W.B.C., 11,500 /㎣ with stab neutrophile 17%, segmentneutrophile 46%, lymphocyte 34%, monocyte 2%, eosinophils 1% and basophil 1%.
Urinalysis; sugar and protein were negative but urobilinogen was positive upto 1 : 80 dilutions of various occasions.
Fecal analysis; non-specific.
Liver function test; Serum bilirubin, total 1. 5 mg. % with direct 0.7 mg% and indirect 0.8 mg%, thymol turbidity test 4.0 units, SGOT 113 units, total protein 6.7 gm/dl. with A/G ratio of 1.1 to 1, alkaline phosphatase 1.3 Bodanski units, fasting blood sugar 100 mg%, and C.C.F. 3+/24 hours. BUN and creatinine were normal.
Roentgenological examination; Chest X-ray was normal.
Serological examination;
V.D.R.L. was negative, Ham´s acid serum test negative, and Donath-Landsteiner test negative. Serum cold agglutinin was tested on numerous occasions as listed below;
Date Cold agglutinin titer
Dec. 12, 1966 1 : 2,560
Dec. 22, 1966 1 : 512
Jan. 3, 1967 1 : 10,320
Jan. ´12, 1967 1 : 32,768
Jan. 16, 1967 1 : 1,048,576
Feb. 20, 1967 1 : 8,388,608
Feb. 25, 1967 1 : 134,217,728
Mar. 8, 1967 1 : 167,000, 000
Cryoglobulin was tested on 4 occasions and results ere all negative.
Macroglobulin was tested on 2 occasions and were own to be negative.
2,00mbs test:Both direct and indirect tests showed strongly positive results.
Immunoelectrophoretic analysis failed to reveal any abnormality as performed at Department of Biochemistry, Catholic Medical College.
Paper electrophoretic study revealed albumin 53. 8% alpha-1 globulin 7.0%, alpha-2 globulin 9.2%, beta globulin 7.7% and gamma globulin 22.3%. Bone marrow examination:
Bone marrow revealed extremely hypercellular, erythrocytic hyperplasia compatible with acute hemolytic anemia. Erythrocytes showed gross clumping even in a CBC-bottle, and typing and crossmatch for blood transfusion were done only with extreme difficull.v. Marked ro ´caux formations were noted on blood smear.
Treatment and Course:
While in this hospital, patient was treated with prednisolone 40 to 80 mg. per day orally and some other non-specific symptomatic medications, and over the ensuing weeks patient´s general conditions improved markedly with improved general feeling and rise of hemoglobin, so that patient could be discharged on 35th hospital day.
A peculiar feature that impressed us was a continuous elevation of cold agglutinin titers despite improved general conditions.
After discharge patient went home to have further careful home-care as directed by us. Despite this fact, patient failed to follow the directions and on several occasions patient exposed himself to cold weather.
Approximately 20 days following discharge patient began to note reappearance of symptoms and therefore he had to be readmitted to Catholic Medical Center, Seoul, Korea on Feb. 21, 1967.
Following second admission, however, patient´s conditions failed to ´improve despite strenuous treawith prednisolone, 6-mercaptopurine and chloroquine, and on 29th day of second hospitalization patient manifested sudden brief episode of chest pain, headache and vomiting, soon followed by coma and death.
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