Systemic capillary leak syndrome: a rare but potentially life-threatening cause of protein loss and oedema in B cell prolymphocytic leukaemia

2020 ◽  
Vol 13 (12) ◽  
pp. e237939
Author(s):  
Rhys Williams ◽  
Dima El-Sharkawi ◽  
Andrew Stewart

A 64-year-old man had a several year history of B prolymphocytic leukaemia (PLL) which behaved indolently and had not required any treatment. Five years after diagnosis, he developed hypoalbuminaemia associated with severe lower-limb oedema, consistent with systemic capillary leak syndrome (SCLS). He recovered spontaneously but went on to have three further increasingly severe and protracted episodes over the subsequent 18 months. There was no identifiable precipitating factor for these episodes, but his peripheral lymphocyte count continued to increase slowly. The start of treatment for his PLL with chemoimmunotherapy was followed by a rapid resolution of residual oedema and normalisation of serum albumin. He has had no further attacks of SCLS in the 14 months since he started therapy for PLL. SCLS is a rare consequence of haematological malignancy which may show an excellent response to treatment of the haematological disease.

2018 ◽  
Vol 5 ◽  
Author(s):  
Riccardo Colombo ◽  
Maddalena Alessandra Wu ◽  
Emanuele Catena ◽  
Andrea Perotti ◽  
Tommaso Fossali ◽  
...  

2014 ◽  
Vol 14 (1) ◽  
Author(s):  
Tadashi Iwasa ◽  
Hiroyuki Ohashi ◽  
Kentaro Kihira ◽  
Yuhki Koike ◽  
Kohei Otake ◽  
...  

2020 ◽  
pp. 004947552097929
Author(s):  
Remya Rajan ◽  
Nitin Kapoor ◽  
Hesarghatta S Asha ◽  
Thomas V Paul ◽  
Nihal Thomas

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.


2021 ◽  
Vol 20 (1) ◽  
pp. 74-77
Author(s):  
Kristen Davies ◽  
◽  
Kirsty Thomas ◽  
Lorna Barton ◽  
Chris Williams ◽  
...  

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.


Rheumatology ◽  
2017 ◽  
Vol 56 (10) ◽  
pp. 1822-1823 ◽  
Author(s):  
Alessandra Meneghel ◽  
Giorgia Martini ◽  
Carolina Birolo ◽  
Alvise Tosoni ◽  
Andrea Pettenazzo ◽  
...  

Immunotherapy ◽  
2021 ◽  
Author(s):  
Ilya Polishchuk ◽  
Alexander Yakobson ◽  
Melanie Zemel ◽  
Adam A Sharb ◽  
Walid Shalata ◽  
...  

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.


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