Brief Report Idiopathic AL amyloidosis and biclonal paraproteinemia: a case report the literature and review of
2001
A case of 79 year-old man slrfferingfrom nephrotic syndrome, infiltrative cardiomyopathy and sensitive neiiropathy of the lower limbs, associated with biclonal gammopathy (IgG K and IgA A), is described. There was a history of non-insulin dependent diabetes mellitirs and of two lung nodules considered as benign lesions on the basis of cytologic, hematologic and instrumental examination. A rectal biopsy positive for amyloid deposition (Congo red histology and immunofluorescence study) led to the diagnosis of AL amyloidosis. Considering that the patient did not fulfill diagnostic criteria for lymphoproliferative diaseases (myeloma, lymphoma or Waldenstrom ’s niacroglubulinemia), nor for secondary maiignantparaproteinemia, a diagnosis of idiopathic AL amyloidosis with biclonal gammopathy was made. Very few cases of idiopathic AL amyloidosis with double component are reported in the literature. Our review suggests that idiopathic AL amyloidosis with biclonal gammopathy is similar to idiopathic AL amyloidosis with monoclonal paraproteinemia in terms of clinical features, response to therapy andprognosis. Further studies, however, are necessary to clarijj the true incidence and the clinical features of idopathic AL amyloidosis associated with biclonal gammopathy.
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