[AL amyloidosis, from diagnosis to treatment]
Camille Villesuzanne1, Arnaud Jaccard2, Martin Nicol3
1Service d'immuno-hématologie, hôpital Saint-Louis, AP-HP, Paris, France.
Abstract:
Al amyloidosis, from diagnosis to treatment. AL amyloidosis is a rare hemopathy characterized by immunoglobulin light chains deposits in almost all organs causing organ failure. The main issue is the early dia¬gnosis, which must be made in front of an unexplained non-specific symptomatology, especially cardiac or renal, in frequently elderly patients with monoclonal gammo¬pathy. Non-invasive biopsies should be made for histolo¬gical confirmation revealing positive congo red and birefringent yellow-green deposits in polarized light specific for amyloidosis. Severity is assessed by biologi¬cal markers of cardiac involvement. The treatment consists in eliminating the plasma or lympho-plasma cell dyscrasia secreting the amyloidogenic light chain, and in proposing supportive care specific to this pathology.
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