cryoglobulinemia
Introduction
Introduction to cryoglobulinemia Cryoglobulinaemia is a series of skin and systemic symptoms caused by cryoglobulin deposition at low temperatures, causing blood circulation disorders. basic knowledge The proportion of illness: 0.015% Susceptible people: no special people Mode of infection: non-infectious complication:
Cause
Cause of cryoglobulinemia
(1) Causes of the disease
Cryoglobulin is a protein that is soluble in cold precipitation and soluble when the temperature rises. It can be classified into three types.
Type I: is a single cell line type, that is, a monoclonal-derived immunoglobulin, and has four types of IgG, IgM, IgA, and condensed solubilized protein.
Type II: is a mixed type consisting of two or more immunoglobulins, one of which is a monoclonal source.
Type III: a polyclonal prototype.
Type I is not resistant to complement, which usually causes macrovascular damage. The mechanism is unknown. Types II and III are mixed.
(two) pathogenesis
Anti-complement, through the activation of the complement system for immune response and tissue damage, mainly leading to small blood vessel damage, abnormally increased cryoglobulin can be divided into primary and idiopathic according to its source, the former is asymptomatic, only found in routine examinations The latter is often associated with immunoproliferative disorders, infectious diseases and the like.
Prevention
Cryoglobulinemia prevention
The pathogenesis of this disease, and the anti-complement activation caused by the action of immune complexes, through the activation of the complement system for immune response and tissue damage, mainly leading to small blood vessel damage. Therefore, it is impossible to prevent the cause of the disease in the clinic. Early detection, early diagnosis and early treatment have a positive effect on the prognosis of the disease. At the same time, the disease often requires long-term use of hormone therapy. It should pay attention to calcium supplementation to avoid aseptic joint necrosis. .
Complication
Cold globulinemia complications Complication
Often associated with immune proliferative disorders, infectious diseases and so on.
Symptom
Symptoms of cryoglobulinemia Common symptoms Skin spots, ecchymosis, swollen papules, blisters or bullae, damage, nodules, reflexes, disappearance, itching, abdominal pain
The most common skin lesions are hemorrhagic purpura, which starts from the lower extremities and gradually spreads to the femur, perineum, buttocks, and a few extend to the upper limbs and oral mucosa. Generally, it does not affect the face and trunk. The rash is characterized by papules, blemishes, and ecchymoses. , small nodules, severe cases may appear blisters, bullae, ulcers, gangrene, conscious itching or burning, severe pain, followed by cold urticaria, Raynaud's phenomenon, reticular bluish, vasospasm, cyanosis Etc. Chronic pigmentation, joint pain is a prominent symptom of patients with mixed cryoglobulinemia, common in the knee joint, kidney damage can be acute, chronic nephritis, and even lead to renal insufficiency or renal failure Death, the nervous system is mainly characterized by peripheral neuropathy as paresthesia, numbness, dyskinesia, sputum reflex disappearance, etc., central nervous system involvement is rare, other visible hepatosplenomegaly, abdominal pain, pericarditis and generalized lymphadenopathy, etc. .
Examine
Examination of cryoglobulinemia
1. Determination of cryoglobulin: The patient's serum is placed at 4 ° C for at least 12 h, and in some cases it may take up to 1 week to precipitate the cryoglobulin for detection. Normal people <10 mg / L, and patients with this disease are often at 250 mg / L or more.
2. Immunoglobulin assay: IgM is often increased, and some IgG and IgA are increased.
3. Complement assay: Type I is normal, and mixed type often decreases in complement.
4. Hematological examination: ESR is often accelerated, abnormal blood clots, and thrombocytopenia.
Histopathology: There was no obvious change in the epidermis. There were eosinophilic amorphous and uniform proteinaceous substances in the small vessels of the dermis and subcutaneous tissues, characterized by fibrin-like necrosis and perivascular pyknosis of neutral polymorphonuclear neutrophils.
Diagnosis
Diagnosis and diagnosis of cryoglobulinemia
diagnosis
According to the clinical manifestations and serum cryoglobulin significantly increased, the disease can be diagnosed. It should be noted that after treatment with corticosteroids, or after the original associated disease is improved, it is difficult to detect cold globulin, and the temporary cold ball should be excluded. Protein increase phenomenon.
Differential diagnosis
1. Condensed agglutinin: It is due to the self-aggregation in the small blood vessels after the high-priced lectin in the serum is cold, and the prominent symptoms are the extremities and the nose, the cyanosis at the ear, accompanied by numbness and pain, and See hemolytic anemia and paroxysmal hemoglobinuria.
2. Cold fibrinogenemia: a protein with condensation in its plasma, clinical manifestations of urticaria, numbness and bleeding in the extremities, which may be primary or secondary to certain malignant diseases.
3. Hand and foot cyanosis: It is due to the spasm of the skin, secondary to the secondary expansion of capillaries and veins, and no spontaneous symptoms.
