[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.
Vella
Francesco Saverio
FS
Sezione di Medicina Interna, Dipartimento di Medicina Interna, Immunologia e Malattie Infettive, Università, Bari. f.vella@intmed.uniba.it
Panella
Elisabetta
E
Masciale
Nunzia
N
Giannelli
Gianluigi
G
Antonaci
Salvatore
S
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