[Clarkson syndrome: a rare clinical condition characterized by generalized edema associated to monoclonal gammopathy].
Generalized edema is commonly due to cardiac failure, renal changes, hepatic and metabolic disturbances, or it can be idiopathic, i.e. primitive lymphedema. Here we describe a patient with several episodes of fluid extravasation characterized by hypotension, hemoconcentration and functional renal insufficiency. These findings, associated to a monoclonal gammopathy, lead to the diagnosis of systemic capillary leak syndrome or Clarkson Syndrome. This rare and perplexing disorder, characterized by a typical three-phase clinical feature, is due to an endothelium permeability alteration, rather responsible of these paroxysmal manifestations. Interleukin-2-pathway is considered as one of the underlying mechanisms. During acute phase the patient underwent therapy with plasma-expanders and glucocorticoids, although in quiescent phase we administered aminophylline, salbutamol and prednisone. After three months, the patient is asymptomatic.
Sezione di Medicina Interna, Dipartimento di Medicina Interna, Immunologia e Malattie Infettive, Università, Bari. f.vella@intmed.uniba.it
Francesco Saverio
FS
Vella
Elisabetta
E
Panella
Nunzia
N
Masciale
Gianluigi
G
Giannelli
Salvatore
S
Antonaci
D000318:Adrenergic beta-Agonists / Q000627:therapeutic use; D000420:Albuterol / Q000627:therapeutic use; D000628:Aminophylline / Q000627:therapeutic use; D000799:Angioedema / Q000175:diagnosis* / Q000276:immunology / Q000503:physiopathology / Q000628:therapy; D000893:Anti-Inflammatory Agents / Q000627:therapeutic use; D001803:Blood Transfusion; D019559:Capillary Leak Syndrome / Q000175:diagnosis* / Q000276:immunology / Q000503:physiopathology / Q000628:therapy; D002199:Capillary Permeability; D002316:Cardiotonic Agents / Q000627:therapeutic use; D004359:Drug Therapy, Combination; D006801:Humans; D007376:Interleukin-2 / Q000378:metabolism; D008297:Male; D008875:Middle Aged; D008998:Monoclonal Gammopathy of Undetermined Significance / Q000175:diagnosis* / Q000276:immunology / Q000503:physiopathology / Q000628:therapy; D011241:Prednisone / Q000627:therapeutic use; D016896:Treatment Outcome
D000318:Adrenergic beta-Agonists; D000893:Anti-Inflammatory Agents; D002316:Cardiotonic Agents; D007376:Interleukin-2; D000628:Aminophylline; D000420:Albuterol; D011241:Prednisone