[Clarkson syndrome: a rare clinical condition characterized by generalized edema associated to monoclonal gammopathy]

Francesco Saverio Vella; Elisabetta Panella; Nunzia Masciale; Gianluigi Giannelli; Salvatore Antonaci

[Clarkson syndrome: a rare clinical condition characterized by generalized edema associated to monoclonal gammopathy]

Recenti Prog Med. 2005 Oct;96(10):488-91.

[Article in Italian]

Authors

Francesco Saverio Vella¹, Elisabetta Panella, Nunzia Masciale, Gianluigi Giannelli, Salvatore Antonaci

Affiliation

¹ Sezione di Medicina Interna, Dipartimento di Medicina Interna, Immunologia e Malattie Infettive, Università, Bari. [email protected]

PMID: 16491771

Abstract

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.

Publication types

Case Reports
English Abstract

MeSH terms

Adrenergic beta-Agonists / therapeutic use
Albuterol / therapeutic use
Aminophylline / therapeutic use
Angioedema / diagnosis*
Angioedema / immunology
Angioedema / physiopathology
Angioedema / therapy
Anti-Inflammatory Agents / therapeutic use
Blood Transfusion
Capillary Leak Syndrome / diagnosis*
Capillary Leak Syndrome / immunology
Capillary Leak Syndrome / physiopathology
Capillary Leak Syndrome / therapy
Capillary Permeability
Cardiotonic Agents / therapeutic use
Drug Therapy, Combination
Humans
Interleukin-2 / metabolism
Male
Middle Aged
Monoclonal Gammopathy of Undetermined Significance / diagnosis*
Monoclonal Gammopathy of Undetermined Significance / immunology
Monoclonal Gammopathy of Undetermined Significance / physiopathology
Monoclonal Gammopathy of Undetermined Significance / therapy
Prednisone / therapeutic use
Treatment Outcome

Substances

Adrenergic beta-Agonists
Anti-Inflammatory Agents
Cardiotonic Agents
Interleukin-2
Aminophylline
Albuterol
Prednisone