Here is my story…

Diagnosed with TNB cancer in 2020, reoccuance in 2021… treated with Keytruda for almost 2 years. I was diagnosed with Addison’s Disease so I stopped Keytruda in July 2023. NED since October 2021… fast forward to January 2024, I started retaining fluid. I had pleurel effusions, abdominal ascites, pelvic fluid, which finally led to pericardial effusion which landed me in the hospital with drain tubes. After 3 months of testing (because it seemed as if they all thought it was cancer returning) they have finally diagnosed me with SCLS due to Keytruda. They have found one study that they are going by but are still uncertain of the right plan of treatment. Right now, the plan is to start IVIg treatments but they don’t really know how often, and possibly start another type of chemo called Avastin. I’m reaching out here in hopes of getting some good feedback. I would love to talk with a doctor that has treated this before as mine aren’t really sure… which makes me nervous. It seems IVIg is the way to go but the protocol is what I’m unclear about… any help is certainly appreciate!!!!

Courtney, welcome to our Community, though as you will see, I don’t think you really belong here!

There is a crucial difference between CLS and SCLS – and it matters because chances are you experienced an instance of the former, but that doesn’t mean that you have SCLS, which is a life-long condition.

Capillary leak syndrome (CLS) is characterized by the mostly one-time escape of blood plasma through capillary walls, from the blood circulatory system to surrounding tissues, muscle compartments, organs or body cavities.

It is a phenomenon most commonly witnessed in sepsis, and less frequently in autoimmune diseases, differentiation syndrome, engraftment syndrome, hemophagocytic lymphohistiocytosis, the ovarian hyperstimulation syndrome, viral hemorrhagic fevers, and snakebite and ricin poisoning.

Pharmaceuticals, including the chemotherapy medications gemcitabine and denileukin diftitox, and the immunotherapy medication pembrolizumab (generic for Keytruda), as well as certain interleukins and monoclonal antibodies, can also cause such capillary leaks.

Systemic capillary leak syndrome (SCLS), also called Clarkson's disease, is an exceedingly rare, grave and episodic medical condition observed largely in otherwise healthy individuals mostly in middle age. It is characterized by self-reversing episodes during which the endothelial cells which line the capillaries, usually of the extremities, separate for one to three days, causing a leakage of plasma mainly into the muscle compartments of the arms and legs. SCLS thus features recurring episodes of CLS.

IVIG has been administered to cancer, sepsis and other patients while experiencing an instance of CLS related to their primary illness or main treatment. However, there is no point in them being put on a regimen of weekly or monthly IVIG unless they happen to experience the pattern of multiple episodes of CLS typical of SCLS.

The medical article that discusses using IVIG for a Keytruda patient is probably this one from 2021: https://www.mcpiqojournal.org/action/showPdf?pii=S2542-4548%2821%2900004-7 It is authored by doctors from the Mayo Clinic, so you could ask your own oncologist to contact them for advice given your situation.

As you will read, however, their patient was only given IVIG when he was experiencing a first episode of Keytruda-induced CLS – but they did not prescribe IVIG to him after he was discharged: “Our patient’s condition quickly stabilized after 5 days of IVIG treatment along with axitinib.”

I wish you no more episodes of CLS and continued success in treating your TNB cancer!

OK!!! All of this is very new to me and I am just trying to find the help I need to feel better!! Thank you for the explanation! I really appreciate it! Your response provided a lot of information that will help me try to navigate through this!!!

I did recieve IVig in the hospital but the fluid still continues so I am not sure my case is a one a done type of deal. I did find a lady that has been experinecing the same thing. She is getting it monthly and is finally seeing a difference after 5 months of treatment. I am praying that is my case! I just had 4900ml drawn off of my abdomen this week. Its been 3 weeks since my last IVig treatment. It has slowed down but hasn't stopped.