Table 2.
Clinical characteristics of the 19 patients presenting with both cryofibrinogen (CF) and cryoglobulins (CG). The levels of CF, CG and plasma fibrinogen (Fg) and immunoglobulin (Ig) composition of plasma and serum cryoprecipitates are also given
| No. | Age (years) | Sex | Diagnosis | Signs or symptoms | CF (g/l) | Fg (g/l) | CG (g/l) | CF | CG | Cold sensitivity | Treatment | Outcome |
|---|---|---|---|---|---|---|---|---|---|---|---|---|
| 1 | 69 | F | Sjögren's disease | Necrotic and purpuric lesions on legs and feet | 4·6 | 4·6 | 5·6 | IgMκ and IgG | IgMκ and IgG | No | Prednisone | Septic shock. Died |
| 2 | 62 | F | Scleroderma (CREST) | Raynaud's phenomenon, skin necrotic ulcerations | 1·7 | 3·5 | 0·26 | 0 | IgG, IgA and IgM | No | Prostacyclin, calcium blockers | Recurrence skin lesions |
| 3 | 76 | F | Polymyositis. Pyocyanic infection | Fever and muscle weakness | 2·8 | 4·6 | 0·01 | IgMλ, IgG and IgA | IgMλ, IgG and IgA | No | Prednisone | Septic shock. Died |
| 4 | 57 | F | Polymyositis | Muscle weakness | 0·32 | 2·9 | 0·06 | IgM | IgM | No | Prednisone | Clinical remission |
| 5 | 34 | M | Systemic lupus erythematosus | Kidney involvement of systemic lupus | 1·4 | 3·1 | 0·1 | IgMλ | IgMλ | No | Cyclophos- phamide, prednisone, aspirin | Clinical remission |
| 6 | 45 | F | Lupus-like arthritis | Arthralgia | 0·12 | 5 | 0·12 | IgG, IgA and IgM | IgG, IgA and IgM | No | Prednisone, cyclophos-phamide | Clinical remission |
| 7 | 37 | F | Hashimoto hypothyroidism | Perineal skin nodules | 3·4 | 3·7 | 0·09 | IgG, IgA and IgM | IgG, IgA and IgM | No | Coumadin, prednisone | Clinical remission |
| 8 | 47 | F | Osteitis | Livedo, ulcerations ofthe legs, gangrene | 3 | 4·6 | 0·08 | IgG, IgA and IgM | IgG, IgA and IgM | No | Coumadin Feet amputation | ND |
| 9 | 50 | M | Erysipelas | Swelling and ulcerations of the lower extremities | 1·3 | 3·6 | 0·01 | IgGκ | IgG, IgA and IgM | No | Antibiotics | CF present only during febrile illness |
| 10 | 29 | M | CMV infection | Weakness, fever, purpuric lesionsof the legs | 0·9 | 2·7 | 0·9 | IgG, IgA and IgM | IgG, IgA and IgM | No | Dressingchanges, wound care | Recurrence during febrile illness |
| 11 | 47 | M | Hepatitis C. Vasculitis. Waldenström | Painful ulcer of the legs. Myocardial infarction | 0·39 | 3·7 | 0·25 | IgMκ, IgG and IgA | IgMκ, IgG and IgA | No | – | Died |
| 12 | 60 | F | Hepatitis C Arthralgia | Purpura of the legs | 0·9 | 4·5 | 0·73 | IgM | IgM | No | IFN-α, ribavirin, plasma-pheresis | Recurrence |
| 13 | 56 | M | Myeloprolifer- ative disease | Mucosal lesions | 0·5 | 5·4 | 0·12 | 0 | IgG | No | Chemo- therapy. IFN-α | Died |
| 14 | 80 | M | Factor XII deficiency | Painful ulcers of the legs Extradural haematoma | 1·28 | 5 | 0·03 | IgG, IgA and IgM | IgG, IgA and IgM | No | Vasodilators | Recurrence of skin lesions |
| 15 | 50 | M | Severe atherosclerosis. Idiopathic? | Non-healing ulcerations on the legs. Alcoholism | 2 | 4·6 | 0·07 | IgG, IgA and IgM | IgG, IgA and IgM | Yes | Occlusive dressing | ND |
| 16 | 35 | M | Idiopathic | Skin necrotic ulcerations, Raynaud's phenomenon, gangrene | 0·49 | 8·6 | 0·07 | IgMλ | IgMλ, IgG and IgA | No | Cyclophos-phamide, prednisone,coumadin | Recurrence of skin lesions |
| 17 | 48 | F | Idiopathic | Recurrent pulmonary embolisms. Renal veinthrombosis | 1·2 | 3·5 | IgMλ | IgMλ, IgG and IgA | No | Heparine, coumadin | Clinical remission | |
| 18 | 52 | M | Idiopathic | Stroke | 0·47 | 5·25 | 0·02 | 0 | IgMλ, IgG and IgA | Yes | Coumadin | Pain of legs and feet |
| 19 | 35 | M | Idiopathic | Purpuric lesions, pulmonary fibrosis | 3·36 | 7·3 | 0·01 | 0 | 0 | No | Prednisone | Purpura |