Publication date: Jan 01, 2025
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?
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| Concepts | Keywords |
|---|---|
| Covid | clarkson’s disease |
| Myeloproliferative | covid-19 |
| Rare | jak 2 mutation |
| Theories | myeloproliferative neoplasm disease |
Semantics
| Type | Source | Name |
|---|---|---|
| disease | MESH | Systemic Capillary Leak Syndrome |
| disease | IDO | entity |
| disease | MESH | edema |
| disease | MESH | hypovolemia |
| disease | MESH | hypovolemic shock |
| disease | MESH | hypoalbuminemia |
| disease | MESH | causes |
| disease | MESH | abnormalities |
| disease | IDO | history |
| disease | MESH | COVID-19 |
| disease | MESH | infection |
| pathway | REACTOME | Reproduction |
| disease | MESH | hematological malignancies |
| disease | MESH | lymphoma |
| disease | MESH | leukemia |
| disease | IDO | protein |
| disease | MESH | complications |
| disease | MESH | acute kidney injury |
| disease | MESH | hepatic failure |
| disease | MESH | ischemic stroke |
| disease | MESH | myocardial infarction |
| disease | MESH | rhabdomyolysis |
| disease | MESH | compartment syndrome |
| disease | MESH | pericardial effusions |
| disease | MESH | septic shock |
| disease | MESH | anaphylaxis |
| disease | MESH | systemic mastocytosis |
| disease | MESH | hereditary angioedema |
| disease | MESH | hemophagocytic lymphohistiocytosis |
| disease | MESH | emergency |
| disease | MESH | syncope |
| disease | MESH | metabolic acidosis |
| drug | DRUGBANK | Creatinine |