Clarkson's syndrome - Capillary leak syndrome

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Clarkson's syndrome - Capillary leak syndrome

Postby patoco » Wed Oct 11, 2006 8:45 am

Clarkson's syndrome - Capillary leak syndrome

Lymphedema People

http://www.lymphedemapeople.com

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Capillary leak syndrome was first described by Clarkson in 1960. It is characterized by recurrent episodes of hypotension with hemoconcentration, hypoalbuminemia without albuminuria and generalized edema.

These episodes are due to capillary hyperpermeability with massive extravasation of plasma containing macromolecules smaller than 200kD and sometimes as big as 900kD. Fifty-seven cases have been reported so far, patients' mean age is 46 years (ranging from 9 to 67 years). Both sexes are equally affected. Monoclonal gammopathy, generally an IgG class, is associated in 82% of cases. For some authors this gammopathy may play a role in pathogenesis, without evidence of amyloidosis. Each episode is often preceded by infection. Pathophysiology is largely misunderstood but cytokines (IL2), leukotrienes and endothelial apoptosis have been suggested to be involved in capillary hyperpermeability.

Therefore, some cases secondary to treatment by recombinant IL2 have been reported. Each episode consists of two phases:

- The initial phase is the capillary leak phase, lasting from 1 to 4 days. Clinical features are abdominal pain, nausea, generalized edema (sometimes responsible for rhabdomyolysis) and hypotension that may result in cardiopulmonary collapse. Acute renal failure is due to acute tubular necrosis consequent to hypovolemia and rhabdomyolysis.

- The second phase results in the recruitment of the initially extravasated fluid. Intravascular overload with polyuria and pulmonary edema often occur. Edema may be more severe due to massive fluid supply in the initial phase. Therefore, monitoring those patients is essential to timely switch to depletion treatment (including diuretics or hemofiltration).

Mortality is reported in 21% of the 57 cases described. However, better management of this condition has recently led to lower mortality. A few prophylactic treatments have been tried with variable results, principally terbutaline and theophyllin. Patients need to be monitored for monoclonal gammopathy since it may turn into multiple myeloma. *Author: Orphanet editorial team (April 2003)*.

http://www.orpha.net/consor/cgi-bin/OC_ ... Expert=188

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A case report of Clarkson's disease: If you don't know it, you'll miss it.

Aug 2006

Bonadies N, Baud P, Peter HJ, Buergi U, Mueller BU.
Department of Internal Medicine, University Hospital Bern, 3010 Bern, Switzerland.

The systemic capillary leak syndrome (SCLS), also known as Clarkson's disease, is a rare disorder characterized by paroxysmal capillary hyperpermeability with a shift of plasma fluid from the intravascular to the interstitial space. A 35-year-old previously healthy woman was admitted with rapidly developing hypovolemic shock syndrome, rhabdomyolysis, and diffuse edema. Laboratory analysis revealed a severe hemoconcentration, renal insufficiency, and paraproteinemia. After exclusion of infection, allergy, burning or drug-induced conditions, the clinical presentation was consistent with the diagnosis of SCLS. Though this is a rare entity, the substantial morbidity and mortality associated with it necessitate the physician's awareness in order to provide timely therapy. This report is meant to enhance awareness of SCLS.

PMID: 16864014 [PubMed - in process]

http://www.ncbi.nlm.nih.gov/entrez/quer ... med_docsum

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Systemic capillary leak syndrome associated with compartment syndrome and rhabdomyolysis.

July 2006

Sanghavi R, Aneman A, Parr M, Dunlop L, Champion D.
Intensive Care Unit and Haematology Department, Liverpool Hospital, University of New South Wales, Australia.

Systemic capillary leak syndrome (SCLS) is a rare disorder characterized by recurrent spontaneous episodes of hypovolaemic shock due to marked plasma shifts from the intravascular to the extravascular space. This presents as the characteristic triad of hypotension, haemoconcentration and hypoalbuminemia often with an associated monoclonal gammopathy. We describe a patient with SCLS who required aggressive fluid resuscitation and emergency fasciotomies for compartment syndrome with rhabdomyolysis. At presentation the patient was considered to have severe erythrocytosis and was therefore initially referred to a haematologist, which appears to be a frequent sequence of presentation for patients with SCLS. This patient also highlights the importance of muscle compartment pressure monitoring during volume resuscitation in patients with SCLS.

PMID: 16802499 [PubMed - indexed for MEDLINE]

http://www.ncbi.nlm.nih.gov/entrez/quer ... med_docsum

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Systemic capillary leak syndrome causes a compartment syndrome of both lower legs and a forearm. A case report with a review of the literature

Sept 2005

Balcarek P, Frosch KH, Quintel M, Sturmer KM.
Unfallchirurgie, Plastische- und Wiederherstellungschirurgie, Georg-August-Universitat, Gottingen. peter.balcarek@web.de

Systemic capillary leak syndrome is a rare disorder which causes recurrent episodes of hypovolemic shock due to a markedly increased plasma shift into the interstitium. Hemoconcentration, hypoalbuminemia and a monoclonal gammopathy are characteristic laboratory findings. A rare manifestation of this disease is a compartment syndrome with rhabdomyolysis and acute renal failure. We describe a patient who suffers from a compartment syndrome of both lower legs and the left forearm secondary to systemic capillary leak syndrome, and discuss the pathophysiological background.

PMID: 15778826 [PubMed - indexed for MEDLINE]

http://www.ncbi.nlm.nih.gov/entrez/quer ... s=15778826

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Systemic capillary leak syndrome presenting remarkable erythrocytosis

Wakao D, Kawai N, Kuwayama Y, Misumi M, Satoh Y, Shimada T, Akiba M, Kishimoto K, Yoshida K, Wakimoto N, Takahashi N, Sugahara Y, Yagasaki F, Itoh Y, Sakata T, Suzuki T, Matsuda A, Bessho M.
First Department of Internal Medicine, Saitama Medical School.

Systemic capillary leak syndrome (SCLS) is a disorder characterized by hypotension, edema, and an increased hematocrit (Ht) due to sudden leakage of plasma into the extravascular space through some unknown mechanism, in which monoclonal gammopathy is observed. A 30-year-old man visited our emergency department because of abdominal pain, and was admitted to our hematology department because of a markedly increased hemoglobin concentration reaching 26.2 g/dl. The polycythemia was thought to be pseudo-polycythemia due to hemoconcentration, and we diagnosed the patient as having SCLS based on the triad of increased hematocrit, whole-body edema which was especially marked in the lower extremities, and monoclonal gammopathy. The patient recovered after administration of extracellular fluids and albumin, but the attacks recurred. Prophylaxis with terbutaline sulfate, theophylline and corticosteroid reduced the frequency of severe attacks. Because there is possibility that patients with SCLS may be admitted to hematology departments due to severe erythrocytosis, we report this case to increase the awareness of hematologists that SCLS is one of the important differential diagnoses of erythrocytosis.

PMID: 11925875 [PubMed - indexed for MEDLINE]

http://www.ncbi.nlm.nih.gov/entrez/quer ... s=11925875

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Systemic capillary leak syndrome.

2002

Ruri CHIHARA1), Hidetomo NAKAMOTO1), Hiroshi ARIMA1), Kenshi MORIWAKI1), Yoshihiko KANNO1), Soichi SUGAHARA1), Hirokazu OKADA1) and Hiromichi SUZUKI1)
1) The Department of Nephrology, Saitama Medical School

[Received: 2001/10/25]
[Released: 2006/03/27]

Abstract:

Systemic capillary leak syndrome (SCLS) is characterized by recurrent hypovolemic shock attributable to increased systemic capillary leakage. A 46-year-old man was admitted to our hospital because of recurrent episodes of generalized edema with hypovolemic shock. Blood laboratory data revealed severe hypoproteinemia with a small monoclonal IgG-κ protein. These findings suggested strongly the diagnosis of SCLS. A regimen with steroid pulse therapy was tried; however, intravascular overloading accompanied by the recruitment of the initially extravasated fluids resulted in acute pulmonary edema. Intensive care and careful monitoring of fluid volume are required in SCLS.
(Internal Medicine 41: 953-956, 2002)

Keywords:
shock, edema, hypoalbuminemia, pulmonary edema

Full Case Study:

http://www.journalarchive.jst.go.jp/eng ... rtpage=953
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