polyclonal cryoglobulinemia
Introduction
Introduction From the immunochemical aspect, cryoglobulin is divided into three types: type I is a monoclonal cold globulin type, contains two or more monoclonal antibodies, including IgA, IgG, and IgM, and type II is monoclonal and multi-type. The cloned mixed cryoglobulin type includes IgM-IgG, IgA-IgG, and IgG-IgG type, and the type III mixed polyclonal cryoglobulin type, including IgA-IgG and IgM-IgG-IgA. Primary cryoglobulinemia mainly includes type II and III cold globulin. The so-called cryoglobulin refers to a protein complex that can undergo reversible agglutination at low temperatures.
Cause
Cause
(1) Causes of the disease
The etiology and pathogenesis of this disease are not yet fully understood. In recent years, it has been found that the incidence of this disease is higher in people infected with hepatitis B virus. However, it has been confirmed that it is an autoimmune rheumatism, and the mixed cold globulin formed by the autoimmune reaction in the body plays a decisive role in its onset. The so-called cryoglobulin refers to a protein complex that can undergo reversible agglutination at low temperatures, and its main component is immunoglobulin, and also contains a small amount of fibrinogen, lipoprotein, collagen, complement and other serum protein components.
(two) pathogenesis
The mechanism of action of cryoglobulin pathogenesis is not fully understood. Studies have shown that cryoglobulin is deposited as an immune complex on the blood vessel wall, activating the classical pathway of complement and the alternative pathway, causing small blood vessels to produce immune vasculitis, resulting in tissue ischemia in the site of the affected blood vessel. At the same time, due to vascular endothelial cell dysfunction and increased blood viscosity, red blood cell agglutination, coagulation and platelet dysfunction, etc., aggravating tissue ischemia, resulting in a series of clinical symptoms. This mechanism may also be the case in diseases such as secondary cryoglobulinemia such as systemic lupus erythematosus. The pathogenic effect of mixed cryoglobulin is also dependent on its cold tolerance and ability to bind complement. The condensable cryoglobulin is easy to deposit at room temperature and acts as a pathogenic agent. It is easy to bind to the mixed immunoglobulin caused by complement. The damage is also strong.
Examine
an examination
Related inspection
Renal CT examination of blood routine urine
In the early stage of the disease, most patients only showed finger or toe Raynaud when they were cold or exposed to cold water. However, in these cases, the typical Raynaud phenomenon of pale-cyan-purple red did not occur, but more cases appeared. Hairpin is the main performance process. As the disease progresses, there may be physical pain and numbness in cold fashion, joint pain or muscle pain, and even purple or purple skin pattern.
1. Skin and mucous membrane damage: The most common type of recurrent thrombocytopenic purpura is recurrent, which is often distributed in the lower extremities, especially around the ankle. It can also be found in other exposed areas such as the nose, ears and mouth. The purpura is intermittent and occurs in batches. Each episode lasts for several days to one week. Most cases can be recurrent for many years. After remission, local pigmentation can be left. Skin ulcers can sometimes occur in skin lesions at the joints such as the ankle.
2. Joints: Polyarticular pain is another common early manifestation of this disease. Any joint in the body can be affected, but the hand and ankle are the most common. Sometimes the onset of symptoms can be accompanied by myalgia, and even mild muscle pain and fatigue can occur in the absence of joint symptoms. The symptoms of joint muscles are sometimes not parallel to the whole condition, and can disappear with the development of the disease. This joint disease often has no obvious morning stiffness.
3. Kidney damage: More than half of the patients have renal involvement, some of which have edema, massive proteinuria and high blood pressure. Most patients with renal impairment present with acute and chronic glomerulonephritis. A small number of patients may develop acute nephritis and even develop uremia. Kidney damage is sometimes asymptomatic. Many patients with kidney damage are found by proteinuria, hematuria, pyuria and red blood cell casts during routine tests or follow-up. Urine protein is mainly albumin, while urine light chain and this - The weekly protein is not high. Therefore, the kidney and its function should be routinely examined during the examination of the disease.
Diagnosis
Differential diagnosis
Primary cold urticaria
The disease is an autosomal dominant hereditary disease, and part of it is acquired. There is neither cold cold blood nor cold agglutinin and cold globulin in the patient's body. The urticaria appears in the body when it is cold, which may be caused by the release of histamine from mast cells due to cold stimulation. The disease does not show joint pain, nephritis and other symptoms during the onset of the disease.
2. Cold hemolysin syndrome
The syndrome is caused by the inclusion of cold hemolysin (which is essentially an IgG antibody) in the body, which binds to red blood cells and causes hemolysis at the temperature below 20 °C. Its clinical features are chills, high fever, headache, abdominal pain, nausea, vomiting, bronchial asthma, hypertension, tachycardia, systemic cold urticaria and soy hemoglobinuria accompanied by acute hemolytic anemia. The Donath-Landsteiner test for the identification of cold hemolysin was positive, usually with a titer of 1:2 to 1:64, and a Coomb test. Generally no joint symptoms, nephritis and other systemic manifestations, the body cold globulin is not high, rheumatoid factor and anti-nuclear antibody negative, normal complement level.
3. Cold agglutinin syndrome
The syndrome is an excessively high concentration of agglutinin in the body, which binds to red blood cells during cold stimulation and causes acute hemolysis syndrome. It has been confirmed by immunological examination that the cold agglutinin belongs to the light chain type IgM antibody and has cold globulin characteristics, usually interacting with the I antigen, and can cause hemolysis under complement mediated action. There are three types of cold agglutinin syndrome: acute, subacute, and chronic. The first two types are secondary to certain viral infections, such as infectious mononucleosis, rubella, and lymphoma. The chronic type is idiopathic.
