Cryoglobulin is precipitated or jelly-like

Introduction Primary cryoglobulinemia is more common in young people and middle-aged people, and women are slightly more than men. When the patient's cold surface temperature is lowered, the cryoglobulin in the extremity blood vessels is precipitated or jelly-like, blocking the capillaries, causing ischemic necrosis and vasospasm in the blood vessel wall, and purpura and cold skin on the skin. Measles is the most common, and some patients may have Raynaud's phenomenon. When the blood contains cold globulin, it is called cold globulin. Cryoglobulin refers to an immunoglobulin which is easy to form a precipitate spontaneously when the temperature is lower than 30 ° C, and is soluble after heating. A class of serum proteins having similar properties such as cold fibrinogen, a complex of C-reactive protein and albumin, and a heparin-precipitating protein are not included.

Cause

(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 and usually causes macrovascular damage, and its mechanism is unknown. Types II and III are all 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 classified into primary and idiopathic according to its source. The former is asymptomatic and found only in routine examinations, the latter often associated with immunoproliferative disorders, infectious diseases, and the like.

an examination

Related inspection

Serum cryoglobulin (CG) serum cryoglobulin

1. General laboratory inspection

ESR is often increased, hemoglobin is reduced, thrombocytopenia, hemagglutination disorder, false positive reaction in laboratory research of sexually transmitted diseases, gamma globulin is increased, rheumatoid factor is often positive and titer is high.

2. gamma globulin

Increase.

3. Immunological examination

Serum complement reduction, especially C4. Rheumatoid factor, antinuclear antibody, cold globulin positive.

4. Determination of cryoglobulin

More than 90% of patients with type I and 80% of type II have a cold globulin content of >1 mg/mL, and more than 80% of type III patients have <1 mg/mL.

5.Ig check

lgM is often elevated, and some patients have increased IgG and IgA.

6. Serum complement

Type I is normal, and patients with mixed cryoglobulinemia often have a decrease in complement.

7. Other

The Coomb test was positive and positive for antinuclear antibodies. Direct immunofluorescence showed Ig, complement and fibrinogen deposition in the vessel wall.

Differential diagnosis

The disease needs to be differentiated from the following diseases:

1. Condensed agglutinin: It is a self-aggregation phenomenon occurring in small blood vessels after the high-priced lectin in the serum is cold, and the prominent symptoms are cyanosis at the extremities and nose and ears, 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. May be primary or secondary to certain malignant diseases.

3. Hand and foot cyanosis: It is due to the spasm of the skin, the secondary hair loss caused by the secondary expansion of the capillaries and veins, and there are no spontaneous symptoms.

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