scholarly journals A Systemic Capillary Leak Syndrome (Clarkson Syndrome) in a Patient with Chronic Lymphocytic Leukemia: A Case Report in an Out-of-Hospital Setting

2016 ◽  
Vol 2016 ◽  
pp. 1-3 ◽  
Author(s):  
Manon Durand Bechu ◽  
Antoine Rouget ◽  
Christian Recher ◽  
Elie Azoulay ◽  
Vincent Bounes
Keyword(s):  
Chronic Lymphocytic Leukemia ◽  
Poor Prognosis ◽  
Hypovolemic Shock ◽  
Hospital Setting ◽  
Capillary Leak Syndrome ◽  
Lymphocytic Leukemia ◽  
Fluid Replacement ◽  
Capillary Leak ◽  
Systemic Capillary Leak Syndrome ◽  
Initial Care

Systemic Capillary Leak Syndrome (SCLS) is a rare disease with poor prognosis, characterized by the occurrence of mucocutaneous and visceral edema with hypotension, hemoconcentration, and unexpected hypoalbuminemia. The disease can be idiopathic (Clarkson syndrome) or secondary to other diseases and treatments. We describe this syndrome in a prehospitalized, 63-year-old patient with chronic lymphocytic leukemia and an idiopathic form of SCLS manifesting as hypovolemic shock. Initial care is hospitalization in intensive care. In addition to etiological treatment if fluid replacement is necessary, treatment must be closely monitored for secondary overload complications. Catecholamine rather than arrhythmogenic support may be associated.

Bilateral simultaneous non-arteritic ischaemic optic neuropathy: a rare complication of idiopathic systemic capillary leak syndrome (SCLS)

2021 ◽  
Vol 14 (5) ◽  
pp. e242847
Author(s):  
Yan Ning Neo ◽  
Manvi Sobti ◽  
Hadi Zambarakji
Keyword(s):  
Optic Nerve ◽  
Visual Field ◽  
Optic Neuropathy ◽  
Capillary Leak Syndrome ◽  
Fluid Replacement ◽  
Visual Field Defects ◽  
Capillary Leak ◽  
Systemic Capillary Leak Syndrome ◽  
Severe Hypotension ◽  
Ischaemic Optic Neuropathy

We report a case of bilateral symmetrical superior visual field defects in a 72-year-old man first reported during the recovery from systemic capillary leak syndrome (SCLS). During the acute illness, he required extensive and prolonged fluid replacement and mechanical ventilation for severe hypotension, shock and multiorgan dysfunction. His visual field defect and optic nerve changes were consistent with a diagnosis of ischaemic optic neuropathy. These remained unchanged over 3 years and he retained excellent 6/7.5 visual acuity bilaterally. We hypothesised the mechanism of bilateral segmental infarction of the optic nerve head to be caused by the hypercoagulable and hypovolaemic state, in addition to pre-existing vascular disease and hypertension. This case highlights the importance of including optic nerve examination in the management plan of SCLS, particularly in individuals with underlying vascular risk factors.

Idiopathic systemic capillary leak syndrome – An often missed diagnosis

2020 ◽  
pp. 004947552097929
Author(s):  
Remya Rajan ◽  
Nitin Kapoor ◽  
Hesarghatta S Asha ◽  
Thomas V Paul ◽  
Nihal Thomas
Keyword(s):  
Hypovolemic Shock ◽  
Capillary Leak Syndrome ◽  
Acute Episode ◽  
Capillary Leak ◽  
Systemic Capillary Leak Syndrome ◽  
Life Threatening ◽  
Missed Diagnosis

Idiopathic systemic capillary leak syndrome (ISCLS) is a potentially fatal disorder characterised by ‘attacks’ of varying intensity of hypovolemic shock in association with haemoconcentration and hypoalbuminaemia. It is a disease of exclusion, and the severity of attacks may mimic sepsis at presentation. We report a case of a lady with recurrent attacks of ISCLS with at least two life-threatening episodes, having been treated elsewhere as a case of steroid insufficiency. The diagnosis is often challenging, and treatment of an acute episode involves the judicious use of fluids and vasopressors, as required. Prophylaxis to prevent further attacks is of varied success.

Idiopathic systemic capillary leak syndrome (Clarkson’s disease) presenting with recurrent hypovolemic shock

2021 ◽  
Vol 20 (1) ◽  
pp. 74-77
Author(s):  
Kristen Davies ◽  
◽  
Kirsty Thomas ◽  
Lorna Barton ◽  
Chris Williams ◽  
...  
Keyword(s):  
Hypovolemic Shock ◽  
Intravenous Immunoglobulins ◽  
Capillary Leak Syndrome ◽  
High Dose ◽  
Capillary Leak ◽  
Systemic Capillary Leak Syndrome ◽  
Compartment Syndromes ◽  
Monthly Basis ◽  
History Of ◽  
Clarkson’S Disease

A 49-year old male with a past medical history of myocardial infarction and compartment syndromes requiring fasciotomies presented on five occasions with hypovolemic shock. We describe his admissions and presumptive diagnoses which required large volumes of intravenous fluids, admission to intensive care for vasopressors and renal replacement therapy. The presentations were always precipitated by a prodrome of fatigue and pre-syncopal episodes. On his last admission, a diagnosis of Idiopathic systemic capillary leak syndrome (ISCLS), also known as Clarkson’s Disease, was reached. He is currently receiving high dose intravenous immunoglobulins on a monthly basis.

scholarly journals A Case Report of Idiopathic Systemic Capillary Leak Syndrome That Occurred During the Postoperative Period of Abdominoperineal Resection for Colorectal Cancer

2015 ◽  
Vol 100 (1) ◽  
pp. 58-62 ◽  
Author(s):  
Tsuyoshi Ozawa ◽  
Hironori Yamaguchi ◽  
Tomomichi Kiyomatsu ◽  
Shinsuke Saito ◽  
Soichiro Ishihara ◽  
...  
Keyword(s):  
Abdominoperineal Resection ◽  
Postoperative Period ◽  
Hypovolemic Shock ◽  
Capillary Leak Syndrome ◽  
Capillary Leak ◽  
Systemic Capillary Leak Syndrome ◽  
Continuous Hemodiafiltration ◽  
Fluid Infusion ◽  
First Case ◽  
Massive Fluid

Abstract A 57-year-old woman without any past medical history underwent abdominoperineal resection for rectal cancer in our department. On postoperative day 15, the patient complained of sudden abdominal pain, and high fever was noted in addition to the appearance of erythema around the stoma. The diagnosis of phlegmon was made, and antibiotic infusion was started. However, a few days later, the patient developed hypovolemic shock with hypoalbuminemia and hemoconcentration. Fasciotomy was performed to exclude the necrotizing fasciitis, though all cultures were negative. Upon exclusion of the differential diagnoses, idiopathic systemic capillary leak syndrome (ISCLS) was diagnosed. She was successfully treated with massive fluid infusion under ventilation and continuous hemodiafiltration. Here, we report the first case of ISCLS that occurred during the postoperative period of colorectal surgery.

scholarly journals Multiglandular Hormone Deficiency in a Patient with Systemic Capillary Leak Syndrome

2015 ◽  
Vol 2015 ◽  
pp. 1-6 ◽  
Author(s):  
Cornelia Then ◽  
Katrin Ritzel ◽  
Christa Seibold ◽  
Johannes F. E. Mann ◽  
Martin Reincke
Keyword(s):  
Hypovolemic Shock ◽  
Intravenous Immunoglobulins ◽  
Primary Hypothyroidism ◽  
Interstitial Space ◽  
Capillary Leak Syndrome ◽  
Hormone Deficiency ◽  
Capillary Leak ◽  
Hormone Binding ◽  
Systemic Capillary Leak Syndrome ◽  
Peripheral Edema

Systemic capillary leak syndrome (SCLS) is a rare but potentially fatal disorder characterized by a loss of fluid and proteins into the interstitial space leading to intravascular hypovolemia up to the point of hypovolemic shock. We report the case of a 64-year-old man with SCLS and multiple hormone abnormalities (primary hypothyroidism, hypoadrenalism, and hypogonadism), deficiency of hormone binding globulins, and hypogammaglobulinemia. The patient was successfully treated with intravenous immunoglobulins, theophylline, and terbutaline. Strikingly, with the dissolution of peripheral edema, hormone levels improved. To our knowledge, this is the first reported case of SCLS associated with polyglandular abnormalities.

scholarly journals Nivolumab induced systemic capillary leak syndrome as an ultra rare life-threatening phenomenon of late toxicity and intravenous immunoglobulin efficacy

Immunotherapy ◽  
2021 ◽  
Author(s):  
Ilya Polishchuk ◽  
Alexander Yakobson ◽  
Melanie Zemel ◽  
Adam A Sharb ◽  
Walid Shalata ◽  
...  
Keyword(s):  
Intravenous Immunoglobulin ◽  
Hypovolemic Shock ◽  
Late Toxicity ◽  
Immunoglobulin Therapy ◽  
Capillary Leak Syndrome ◽  
High Dose ◽  
Capillary Leak ◽  
Systemic Capillary Leak Syndrome ◽  
Intravenous Immunoglobulin Therapy ◽  
Life Threatening

Systemic capillary leak syndrome (SCLS) is a life-threatening disease. It is characterized by severe capillary hyperpermeability to proteins resulting in hemoconcentration, hypoalbuminemia and hypovolemic shock. Its treatment remains supportive, and the prognosis is generally poor. We report on a 51-year old male with melanoma treated with nivolumab for 1 year. 1 month following the completion of the treatment, the patient presented with signs of hypovolemic shock, anasarca, hemoconcentration and hypoalbuminemia. After excluding other diseases, a diagnosis of nivolumab-induced systemic capillary leak syndrome was made. A high dose of intravenous steroid therapy was promptly initiated without any significant clinical improvement. Intravenous immunoglobulin therapy was then administered with normalization of blood pressure, hemoconcentration and complete resolution of anasarca. Intravenous immunoglobulin should be considered a first-line treatment option for this rare phenomenon.

scholarly journals Systemic Capillary Leak Syndrome as an Initial Presentation of ALK-Negative Anaplastic Large Cell Lymphoma

2012 ◽  
Vol 2012 ◽  
pp. 1-4 ◽  
Author(s):  
Laura S. Lourdes ◽  
Samer Z. Al-Quran ◽  
Nam H. Dang ◽  
Merry-Jennifer Markham
Keyword(s):  
Anaplastic Large Cell Lymphoma ◽  
Cell Lymphoma ◽  
Hypovolemic Shock ◽  
Large Cell ◽  
Capillary Leak Syndrome ◽  
Capillary Leak ◽  
Systemic Capillary Leak Syndrome ◽  
Initial Manifestation ◽  
Antineoplastic Treatment ◽  
Large Cell Lymphoma

Systemic capillary leak syndrome (SCLS) is a rare disease characterized by third spacing of plasma into the extravascular compartment, leading to anasarca, hemoconcentration, and hypovolemic shock. It has been rarely associated with lymphomas, and reports usually indicate that it occurs after antineoplastic treatment. We present the case of a patient with ALK-negative anaplastic large cell lymphoma who presented with SCLS as the initial manifestation of her lymphoma. The SCLS resolved with treatment of the malignancy with steroids and chemotherapy.

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