Overview.
Cryofibrinogenemia is a rare disorder in which cold-precipitated fibrinogen is present in the plasma. The main symptoms of the disease include cold allergy symptoms, bleeding, thrombosis and ulcers. Cryofibrinogenemia can be divided into two categories: primary and secondary. Primary cryofibrinogenemia is currently of unknown cause, is uncomplicated by other diseases, and is clinically rare. Secondary cryofibrinogenemia can occur in the course of many diseases.
Causes
1. Primary cryofibrinogenemia
Primary cryofibrinogenemia has no known cause, is uncomplicated, and is rare.
2. Secondary Cryoglobulinemia
Secondary cryofibrinogenemia can be secondary to the following diseases.
(1) Malignant tumors: prostate cancer, lung cancer, gastric cancer, ovarian cancer, bladder cancer, pancreatic cancer and liver cancer, etc.; other malignant tumors, such as multiple myeloma and chronic lymphocytic leukemia. The incidence of cold fibrinogenemia in metastatic cancer is higher than that in non-metastatic cancer.
(2) Connective tissue diseases and certain skin diseases: e.g. systemic lupus erythematosus, systemic sclerosis, rheumatoid arthritis, erythema nodosum, Behçet’s syndrome, etc.
(3) Others: atypical pneumonia, meningitis. There are also myocardial infarction, cirrhosis, gout, ulcerative colitis, etc. can appear cold fibrinogenemia.
Symptoms
1. Thrombosis and embolism
Traveling thrombophlebitis, swelling of limbs, myocardial infarction, pulmonary infarction, cerebral infarction, aortic thrombosis.
2.Cold allergy
Cold urticaria, marble-like skin lines, cyanosis of limbs, numbness of limbs, hands and feet, etc.
3.Bleeding
Bleeding symptoms mainly include epistaxis, hemoptysis, hematemesis, skin purpura, petechiae, subnail hemorrhage, gastrointestinal bleeding, hematuria and recurrent hip hematoma.
4. Ulcer and gangrene
Mainly lower limb ulcers, some patients may only have finger (toe) gangrene or extensive necrosis.
Examination
Anemia, rapid blood sedimentation, increased white blood cell count, positive blood cold fibrinogen assay.
Diagnosis
Patients with cold intolerance, unexplained thrombosis and hemorrhage should have a positive serum cryofibrinogen assay to confirm the diagnosis of the disease.
Differential diagnosis
This disease should be differentiated from the following diseases.
1. Cryoglobulinemia
Increased serum cryoglobulin content, mainly manifested as clinical symptoms of vasculitis, i.e., papules, purpura, petechiae, necrosis, blisters, blood blisters or ulcers. Serum cold precipitation test is positive.
2. Cold agglutinin syndrome
There are hemorrhagic phenomena and wandering thrombophlebitis, but hemoglobinuria and hemolytic anemia may occur in severe cases. Cell agglutination can be seen in the cold agglutination test.
Treatment
1. General treatment
Protect from cold and keep warm, avoid fatigue, and treat the original disease.
2.Drug treatment
Anticoagulant heparin is the drug of choice for this disease, which can inhibit the formation of cold fibrinogen and prevent the formation of thrombus.