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To describe a new autoinflammatory syndrome with recurrent fever and monoclonal gammopathy that differs from Schnitzler syndrome.We conducted a retrospective study of patients with monoclonal gammopathy and recurrent fever of unknown A case of IBL-like T cell lymphoma with serum monoclonal gammopathy was reported. A 58-year-old woman, who had suffered from heart failure, was admitted because of asthma attack, fever and lymphadenopathy. Leucopenia with a small amount of atypical lymphocytes was detected. Serum analysis showed
BACKGROUND
Schnitzler syndrome (SS) is a rare autoinflammatory disorder characterized by a recurrent urticarial rash and a monoclonal immunoglobulin M gammopathy, as well as 2 of the following minor criteria: recurrent fever (>38°C), objective signs of abnormal bone remodeling, elevated C-reactive
A 65 years old man has been suffering from the upper limbs and legs for 2 years. Bone pain was associated with a non pruritic chronic urticaria and fever. Bone scintigraphy showed lesions of the lower limbs. X rays showed only one sclerotic bone lesion. Immunoelectrophoretic analysis showed an IgM
Schnitzler syndrome describes the simultaneous occurrence of monoclonal gammopathy and chronic urticaria with at least two additional minor symptoms (arthralgia, bone pain, fever of uncertain origin, hepato- or splenomegaly, lymphadenopathy, increased erythrocyte sedimentation rate,