# A Case Report of Systemic Capillary Leak Syndrome: When More Than One Inciting Factor Exists, the Question Is Who Pulls the Trigger?

## TL;DR

This paper presents a rare case of a man with a condition called systemic capillary leak syndrome who also had a blood-related cancer, raising questions about what caused the syndrome.

## Contribution

This is the first reported case of systemic capillary leak syndrome coexisting with a myeloproliferative neoplasm.

## Key findings

- The patient had idiopathic systemic capillary leak syndrome and a myeloproliferative neoplasm.
- The case raises uncertainty about which condition triggered the systemic capillary leak syndrome.
- The patient had a history of previous immunization and a prior COVID-19 infection.

## Abstract

Systemic capillary leak syndrome (SCLS) constitutes a rare clinical entity. It is characterized by spontaneous, recurrent episodes of increased capillary permeability, leading to a double clinico-biological paradox: diffuse pitting edema with hypovolemia or hypovolemic shock, and hemoconcentration with hypoalbuminemia, in the absence of secondary causes for such abnormalities. Even though several theories have been proposed, the exact pathophysiology of SCLS remains unclear. We report herein a case of idiopathic SCLS in a 38-year-old male patient with a history of previous immunization, following COVID-19 infection, who was also diagnosed with a myeloproliferative neoplasm (MPN). To our knowledge, this is the first case where SCLS coexists with an MPN, leading to a legitimate question: Who pulls the trigger?