Primary cryoglobulinemia is more common in young people and middle-aged people, and is slightly more common in women than in men. When the patient’s body surface temperature decreases in cold, cold globulin in the extremity vascular precipitation or jelly, blocking the capillaries and causing ischemic necrosis and vasospasm of the vessel wall, skin purpura and cold urticaria are the most common, and some patients can appear Raynaud’s phenomenon.
The most common skin damage is hemorrhagic purpura, which starts from the lower extremities and gradually extends to the femur, perineum and buttocks, and a few extends to the upper extremities and oral mucosa, usually not involving the face and trunk. The rash is characterized by papules, petechiae, petechiae, small nodules, and in severe cases, blisters, macules, ulcers, and gangrene. Itching or burning may be conscious, and in severe cases, pain may be present. Next, cold urticaria, Raynaud’s phenomenon, reticular cyanosis, vasospasm of the extremities, and cyanosis are seen. Hyperpigmentation is seen in chronic cases. Arthralgia is a prominent symptom in patients with mixed cold globulinemia and is commonly seen in the hand and knee joints. Renal damage may manifest as acute or chronic nephritis, and may even lead to renal insufficiency or renal failure and death. The main manifestations of the nervous system are peripheral neuropathy manifested as abnormal sensation, numbness, motor impairment, and loss of tendon reflexes. Central nervous system involvement is rare. Other symptoms include hepatosplenomegaly, abdominal pain, pericarditis, and generalized lymph node enlargement.
The diagnosis of the disease is based on clinical manifestations and a significant increase in serum cryoglobulin. It should be noted that cryoglobulins are not easily detected after treatment with corticosteroids or after the improvement of the existing concomitant diseases. A temporary increase in cryoglobulins should also be excluded.
How to effectively prevent the precipitation or jelly-like appearance of cryoglobulins?
(A) Treatment Treat the primary disease, avoid cold, and pay attention to keep warm. Various treatment methods are temporary symptomatic treatment. Plasma replacement method: to remove immunoglobulins or immune complexes and rapidly reduce circulating cold globulins to relieve cold-excited symptoms. Corticosteroids have varying degrees of efficacy in controlling fever, skin lesions, and arthralgia. The dose should be equivalent to prednisone 30-50 mg/d. Immunosuppressants: maintain circulating cold globulin levels without elevating them through toxic effects on dividing cells. Penicillamine: It cleaves the disulfide bonds in immunoglobulins, thereby depolymerizing IgM and affecting immunoglobulin synthesis, but its clinical efficacy is not yet certain. Interferon: It can be used to treat mixed cryoglobulinemia.
Chinese medicine: warming the meridians, activating blood circulation and relieving pain. Warming the meridians and Tongluo Tang plus reduction.
(II) Prognosis Often associated with immunoproliferative disorders, infectious disorders, etc.