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

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.

Low hormone levels during an attack of systemic capillary leak syndrome normalizing after treatment

We report herein the case of a patient with low blood levels of different hormones during a systemic capillary leak syndrome (Clarkson’s disease) attack.

Capillary leak syndrome (CLS) from rituximab therapy of lymphoma

Capillary Leak Syndome (CLS) is characterized by plasma extravasation into the interstitium with resultant hypotension, anasarca, hemoconcentration, and hypoalbuminemia in the absence of albuminuria. Initially reported in Clarkson’s disease (systemic capillary leak syndrome, SCLS), CLS has been observed in multiple disease settings, the most common being sepsis. In Oncology, CLS has been reported more often as a complication from therapy, and less often from malignancy. In this case study, we documented clinical manifestation, laboratory features and radiological findings of CLS from rituximab therapy when employed in combination with a multi-agent chemotherapy regimen (EPOCH-R). Differentiating drug-induced CLS from sepsis, which presents with the same clinical features, is important in avoiding further exposure to rituximab, which could be fatal to the patient.

Capillary Leak Syndrome From Rituximab Therapy of Lymphoma

Capillary leak syndrome (CLS) is characterized by plasma extravasation into the interstitium with resultant hypotension, anasarca, hemoconcentration, and hypoalbuminemia in the absence of albuminuria. Initially reported in Clarkson’s disease (systemic capillary leak syndrome), CLS has been observed in multiple disease settings, the most common being sepsis. In oncology, CLS has been reported more often as a complication from therapy, and less often from malignancy. In this case study, we documented clinical manifestation, laboratory features, and radiological findings of CLS from rituximab therapy when employed in combination with a multi-agent chemotherapy regimen (EPOCH-R). Differentiating drug-induced CLS from sepsis, which presents with the same clinical features, is important in avoiding further exposure to rituximab, which could be fatal to the patient.

Ovarian hyperstemulation syndrome as a private case of iatrogenic capillary leak syndrome

Idiopathic capillary leak syndrome was first described in 1960 and later received its name in honor of the discoverer - Clarkson's Disease. However, in the past two decades, more cases have been reported than in the previous 35 years, most likely due to improved recognition, the widespread use of assisted reproductive technologies in the treatment of infertility and the emergence of targer therapy - monoclonal antibodies for the treatment of cancer and autoimmune diseases. Except for Clarkson's disease, capillary leak syndrome can occur in engraftment syndrome, differentiation syndrome after bone marrow transplantation, ovarian hyperstimulation syndrome, hemophagocytic lymphohistiocytosis, viral infections, mostly hemorrhagic fevers, some autoimmune diseases, administration of recombinant interleukins, cytostatics, some monoclonal antibodies including treatment with the latest anticancer drugs - immune checkpoint inhibitors, snakebite envenomation, and ricin poisoning. This syndrome is characterized by a sharp increase in vascular permeability for fluid and protein molecules that leads to the loss of fluid into the interstitium, and presents with acute onset of severe edema, often asymmetric, hypotension, polyserositis, hemoconcentration and low blood protein level. In assisted reproductive technology programs, the introduction of gonadotropins results in an increase in estradiol levels, which leads to the development of immunopathological process, accompanied by leukocyte infiltration of blood vessels and secondary hypercytokinemia. Subsequently, the secondary «wave» of cytokines and vasoactive substances secreted in response to the introduction of human chorionic gonadotropin as an ovulation trigger, leads to disruption of interendothelial contacts and the development of ovarian hyperstimulation syndrome. It is necessary to conduct a further study of the pathogenesis of capillary leak syndrome, in order to develop promising methods for the prevention and correction of ovarian hyperstimulation syndrome in assisted reproductive technology programs.