Chlorambucil treatment of patients with cold agglutinin syndrome.
نویسندگان
چکیده
T HE COLD AGGLUTININ SYNDROME (C.A.S.) is characterized by high-titre cold agglutinins, a hemolytic anemia, Raynaud phenomena in the cold, various degrees of hemoglobinuria, and sometimes gangrene. Because of the hemolysis, the syndrome is most often classified as an autoimmune hemolytic anemia. However, treatment with ACTH, corticosteroids or splenectomy, most often used in this condition, has been without effect.’ It is now generally agreed that the primary pathogenic factor in the desease is the presence of monoclonal IgMK globulins2 causing agglutination of red cells at temperatures below the normal human body temperature. In many respects the C.A.S. is related to Waldenstr#{246}m’s macroglobulinaemia although the concentration of the 1gM M-component in this disease is usually higher, and the 1gM molecules do not agglutinate red cells in the cold. Patients with C.A.S. living in a temperate climate are often prevented from working or moving outdoors in the winter time, and death caused by serious attacks of hemoglobinemia has been reported. For this reason, four patients have been treated with chlorambucil ( Leukeran#{174}, NN-Di-2chlorethyl-p-aminophenyl-butyric acid). This cytostatic agent reduces the concentration of 1gM in macroglobulinemia Waldenstr#{246}m3 and, as will be seen, has a similar effect in C.A.S.
منابع مشابه
Chronic Hemolytic Anemia Due to Cold
A male, 47, developed chronic hemolytic anemia in association with hi9h titers of cold agglutinins in 1 966. His symptpm of the cold agglutinin syndrome began in 1 951 with cyanosis of the extremities nd face after exposure to cold. In 1 956, hemoglobinuna was first noted after chilling. In 1 962, the cold agglutinin titer was 1 : 1 6,000, and his red cells were coated with complement globulins...
متن کاملRefractory cold agglutinin-immunohaemolytic anaemia associated to marginal zone lymphoma responding to rituximab.
Cold agglutinin immunohaemolytic anaemia (CAIA) responds poorly to standard treatment. We report a case of marginal zone lymphoma complicated by CAIA that responded to rituximab after failing to respond to corticosteroids and chlorambucil.
متن کاملA case report of perioperative managements for a patient with gastric cancer and cold agglutinin syndrome
RATIONALE Gastric cancer patient with cold agglutinin syndrome (CAS) is an extremely rare entity. This kind of patients is very sensitive to the environment, and they always need scrupulous perioperative treatment, however the experience of perioperative treatment for these patients has been seldom reported. PATIENT CONCERNS The patient was a 54-year-old male. He suffered diarrhea for 3 month...
متن کاملMeasurement of the third component of complement bound to red blood cells in patients with the cold agglutinin syndrome.
The amount of the third component of complement (C3) bound to red cells of patients with the cold agglutinin syndrome was determined by a quantitative assay, measuring the fixation of the first component of complement by anti-C3. Abrupt reduction in the serum concentration of cold agglutinin by plasmapheresis markedly decreased the hemolytic rate, but the amount of C3 bound to circulating cells...
متن کاملذخیره در منابع من
با ذخیره ی این منبع در منابع من، دسترسی به آن را برای استفاده های بعدی آسان تر کنید
برای دانلود متن کامل این مقاله و بیش از 32 میلیون مقاله دیگر ابتدا ثبت نام کنید
ثبت ناماگر عضو سایت هستید لطفا وارد حساب کاربری خود شوید
ورودعنوان ژورنال:
- Blood
دوره 35 1 شماره
صفحات -
تاریخ انتشار 1970