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

Abstract

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.

Capillary leak syndrome with immune checkpoint inhibitors and anti-cancer targeted therapy agents: Not that common

“…1,3,5,12,13,16 It is possible that in Rituximab the cytokine release is mediated by two mechanisms: (A) Fc of CD20 interacting with Fcγ receptors on macrophages and (B) apoptosis induced by damage to calcium channels on CD20 antigen. 6 Cell mediated autotoxicity may play a role in IC associated CLS. Beyond humoral mediators, T-CD8 cells may also be involved because these cells surround damaged endothelial cells in CLS.…”

Acute onset facial oedema: an unusual presentation of acute lymphoblastic leukaemia in a child

“…In the context of a normal serum albumin and negative urine protein, diagnostic suspicion turns to mediastinal pathology causing poor lymphatic or venous drainage; however, this was ruled out with further imaging studies in this case. Capillary leak syndrome is a rare phenomenon that can occur due to haematological malignancy14 15 in which oedema results from hypercytokinaemia and endothelial dysfunction. However, this condition typically manifests as systemic oedema and hypoalbuminaemia which was not seen in our patient.…”

Idiopathic Systemic Capillary Leak Syndrome: A Rare Cause of Refractory Shock Following Influenza B Infection

“…Triggers may include surgical interventions, malignancies, medications, and viral infections. 3 , 17 – 19 Influenza A is perhaps the most common viral ISCLS trigger, yet less than a handful of cases have been reported. 20 – 22 To the best of our knowledge this is the first report of influenza B precipitating an ISCLS attack.…”