cold agglutinin syndrome
Introduction
Introduction to cold agglutinin syndrome Cold agglutinin syndrome is a group of diseases characterized by chronic hemolytic anemia and microcirculatory embolism due to autoreactive erythrocyte agglutination and cold-induced factors. basic knowledge Sickness ratio: 0.05% Susceptible people: no special people Mode of infection: non-infectious Complications: anemia
Cause
Cause of cold agglutinin syndrome
For any reason, it is idiopathic cold agglutinin syndrome. Secondary cold agglutinin syndrome is found in diseases such as schistosomiasis, filariasis, malaria, African trypanosomiasis, cirrhosis, atypical pneumonia, SLE, and hemolytic anemia.
There are three cases of this disease:
Acute type (20%):
The condensation agglutination titer is as high as 1:64 million, which is found in certain viral diseases such as infectious mononucleosis, rubella, jaundice, idiopathic pericarditis, and the like, especially atypical pneumonia.
Other (10%):
Unexplained idiopathic cold agglutinin disease occurs in the elderly and manifests as cyanosis of the extremities, hemolytic anemia, and hematuria. This type of condensation agglutination titer can be as high as 1: 1 million (at 0 ° C).
Subacute type (20%):
There are also similar titers to the acute type, found in certain lymphomas.
Prevention
Cold agglutinin syndrome prevention
Keeping warm is the only reliable and effective prevention and treatment method for this disease.
Complication
Complications of cold agglutinin syndrome Complications anemia jaundice
Can be combined with mild to moderate anemia, partially combined with jaundice.
Symptom
Symptoms of cold agglutination syndrome common symptoms jaundice
In the cold environment, the ear is auspicious, the tip of the nose, and the fingers are cyanotic, but once it is warmed, it disappears. Chronic mild to moderate anemia, may have jaundice, secondary to infection in patients with cold agglutinin syndrome, hemolysis is often self-limited and slightly .
The concentration of cold agglutinin is significantly increased, combined with clinical manifestations, can be diagnosed as cold agglutinin syndrome.
Examine
Examination of cold agglutinin syndrome
Blood, bone marrow and laboratory tests for hemolysis meet the changes of hemolytic anemia. There are no red blood cell malformations in the peripheral blood, and the cold agglutinin test is positive. The titer can reach 1:1000 or higher at 4 °C, and albumin at 30 °C. Or the serum lectin titer is still relatively high in diagnostic value, anti-human globulin direct test positive, almost all C3 type.
Diagnosis
Diagnosis and identification of cold agglutinin syndrome
Differential diagnosis
1. Raynaud syndrome (Raynaud) This disease may occur in the hand and foot due to arterial spasm of the extremities, but the disease does not appear in the cold season, there is pale and reactive congestion before the occurrence of cyanosis, the nose and the ear wheel do not cyanosis, serious There may be local gangrene, and both the cold agglutination test and the Coombs test are negative.
2. cryoglobulinemia cryoglobulin is an abnormal plasma protein, mostly IgM, can occur in a variety of diseases including plasma cell myeloma, lymphoma, systemic lupus erythematosus, cryoglobulinemia patients Due to the increase of plasma viscosity, causing obstruction of peripheral blood vessels, fingertip cyanosis may occur, but both the condensation set test and the Coombs test may be negative.
3. Patients with paroxysmal cold hemoglobinuria may have hemolysis and anemia, but the hot and cold hemolysis test (Donath-Landsteiner test, DL test) is positive, while the cold agglutination test is negative.
