TITLE

Idiopathic Systemic Capillary Leak Syndrome (Clarkson's Disease): The Mayo Clinic Experience

AUTHOR(S)
Kapoor, Prashant; Greipp, Patricia T.; Schaefer, Eric W.; Mandrekar, Sumithra J.; Kamal, Arif H.; Gonzalez-Paz, Natalia C.; Kumar, Shaji; Greipp, Philip R.
PUB. DATE
October 2010
SOURCE
Mayo Clinic Proceedings;Oct2010, Vol. 85 Issue 10, p905
SOURCE TYPE
Academic Journal
DOC. TYPE
Article
ABSTRACT
OBJECTIVE: To determine clinical features, natural history, and outcome of a well-defined cohort of 25 consecutive patients with idiopathic systemic capillary leak syndrome (SCLS) evaluated at a tertiary care center. PATIENTS AND METHODS: Records of patients diagnosed as having SCLS from November 1, 1981, through April 30, 2008, were reviewed. Descriptive statistics were used to analyze patient demographics, clinical features, complications, and therapeutic interventions. RESULTS: Of the 34 patients whose records were reviewed, 25 fulfilled all diagnostic criteria for SCLS. The median age at diagnosis of SCLS was 44 years. Median follow-up of surviving patients was 4.9 years, and median time to diagnosis from symptom onset was 1.1 years (Interquartile range, 0.5-4.1 years). Fluilke illness or myaigia was reported by 14 patients (56%) at onset of an acute attack of SCLS, and rhabdomyoiysis developed in 9 patients (36%). Patients with a greater decrease in aibumin level had a higher likehood of developing rhabdomyoiysis (P=.03). Monoclonai gammopathy, predominantly of the lgG-K type, was found. In 19 patients (76%). The progression rate to multiple myeioma was 07% per person-year of follow-up. The overall response rate to the different therapies was 76%, and 24% of patients sustained durable (>2 years) complete remission. The estimated 5-year overaii survival rate was 76% (95% confidence intervai, 59%-97%). CONCLUSION: Systemic capillary leak syndrome, a rare disease that occurs in those of middle age, is usually diagnosed after a considerable delay from onset of symptoms. The degree of albu- min decrement during an attack correlates with development of rhabdomyoiysis. A reduction in the frequency and/or the severity of attacks was seen in nearly three-fourths of patients who were offered empirical therapies. The rate of progression to multiple myeioma appears to be comparable to that of monocional gammopathy of undetermined significance.
ACCESSION #
54592672

 

Related Articles

  • Incorporation of the bone marker carboxy-terminal telopeptide of type-1 collagen improves prognostic information of the International Staging System in newly diagnosed symptomatic multiple myeloma. Jakob, C.; Sterz, J.; Liebisch, P.; Mieth, M.; Rademacher, J.; Goerke, A.; Heider, U.; Fleissner, C.; Kaiser, M.; von Metzler, I.; Müller, C.; Sezer, O. // Leukemia (08876924);Sep2008, Vol. 22 Issue 9, p1767 

    Several prognostic markers, including parameters of tumor burden and cytogenetics, were adopted to identify high-risk patients in multiple myeloma (MM). Recently, the International Staging System (ISS), including β2-microglobulin (β2M) and albumin, was introduced for patients with...

  • Correction.  // Clinical Journal of Oncology Nursing;Feb2014, Vol. 18 Issue 1, p15 

    A correction is presented to the article "Monoclonal Gammopathy of Undetermined Significance--Making It Understandable to Patients," by P. Rule and J.M. Brant from the December 2013 issue of the journal on the subject of the authors' disclosures.

  • New studies.  // MLO: Medical Laboratory Observer;Oct2009, Vol. 41 Issue 10, p6 

    The article offers information related to medical studies. It notes that a study published in the August 2009 issue of "Mayo Clinic Proceedings" revealed that monoclonal gammopathy of undetermined significance (MGUs) is not a precursor of serious diseases. The Sweden's Lund University...

  • Swelling of the left hand and lower arm. Singer, Karl; DiLoreto, Stacy // Patient Care;6/15/2000, Vol. 34 Issue 11, p173 

    Reports on a case of axillary vein thrombosis. Diagnosis and treatment of, and mortality rate for, the disease; Swelling in the left subclavian vein of an 87-year-old man; History of benign monoclonal gammopathy.

  • Monoclonal Gammopathy of Undetermined Significance. Fiegl, Michael; Greil, Richard // New England Journal of Medicine;6/27/2002, Vol. 346 Issue 26, p2087 

    A letter to the editor is presented in response to the article "A Long-Term Study of Prognosis in Monoclonal Gammopathy of Undetermined Significance" in the February 21, 2002 issue.

  • Monoclonal Gammopathy of Undetermined Significance. Montoto, Silvia; Bladé, Joan; Montserrat, Emili // New England Journal of Medicine;6/27/2002, Vol. 346 Issue 26, p2087 

    A letter to the editor is presented in response to an article on monoclonal gammopathy, published in a previous issue.

  • Monoclonal Gammopathy of Undetermined Significance. Kyle, Robert A.; Rajkumar, S. Vincent; Melton, Joseph // New England Journal of Medicine;6/27/2002, Vol. 346 Issue 26, p2087 

    A response by Robert A. Kyle and colleagues to a letter to the editor about their article on monoclonal gammopathy, published in a previous issue is presented

  • Monoclonal Gammopathy of Undetermined Significance. Minisola, Salvatore; Pepe, Jessica; Romagnoli, Elisabetta // New England Journal of Medicine;5/24/2007, Vol. 356 Issue 21, p2223 

    This article presents a letter to the editor in response to "Monoclonal gammopathy of undetermined significance," by J. Blad� in the December 28, 2006 issue.

  • Monoclonal Gammopathy of Undetermined Significance. Blad�, Joan // New England Journal of Medicine;5/24/2007, Vol. 356 Issue 21, p2223 

    This article presents a letter to the editor in response to "Monoclonal gammopathy of undetermined significance," by J. Blad� in the December 28, 2006 issue.

Share

Read the Article

Courtesy of VIRGINIA BEACH PUBLIC LIBRARY AND SYSTEM

Sorry, but this item is not currently available from your library.

Try another library?
Sign out of this library

Other Topics