{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,27]],"date-time":"2026-09-27T05:10:12Z","timestamp":1790485812854,"version":"4.1.0"},"reference-count":20,"publisher":"MDPI AG","issue":"11","license":[{"start":{"date-parts":[[2018,11,6]],"date-time":"2018-11-06T00:00:00Z","timestamp":1541462400000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["JCM"],"abstract":"<jats:p>Systemic capillary leak syndrome (SCLS) is a rare disease characterized by shock caused by capillary hyperpermeability. The disease can occur in cancer patients and effective therapeutic strategies have not been established yet. The aim of the study was to analyze the clinical and laboratory data, treatment modalities, and mortality rate of patients and to identify contributing factors leading to mortality of SCLS in cancer. We searched MEDLINE (inception to July 2018) and of 4612 articles, we identified 62 case reports on SCLS associated with cancer or cancer-related drugs in a total of 53 articles. SCLS was associated with cancer itself in 43.6%, with anti-cancer agents in 51.6% and bone marrow transplantation (BMT) in 4.8%. Among anti-cancer agents, granulocyte-colony stimulating factor (G-CSF) was the most frequently associated drug (14.6%), followed by interleukin (IL)-2 (11.4%). The most common associated malignancies were hematologic (61.3%) with non-Hodgkin lymphoma (22.7%) and multiple myeloma (12.9%) being the leading causes. Common symptoms and signs included dyspnea (27.4%), edema (67.7%), hypotension (32.2%), pleural effusion (29.0%), ascites (22.7%), oliguria (22.7%), and weight gain (21.0%). Patients with SCLS were treated with steroids (59.7%), volume replacement (33.8%), diuretics (24.2%), inotropes (9.6%), methylxanthines (12.8%), \u03b22 agonists (4.8%), while intravenous immunoglobulins (IVIG) were administered in 2 patients (3.2%) only. Among sixteen deaths during follow-up, four were directly attributed to SCLS. Hematologic malignancies were associated with an increased risk for mortality (hazard ratio (HR) 8.820, 95% confidence interval (CI) 1.126\u201369.063, p = 0.038). Taken together, SCLS can be one important adverse event in cancer patients and careful monitoring of fluid volume is required in the management of SCLS.<\/jats:p>","DOI":"10.3390\/jcm7110418","type":"journal-article","created":{"date-parts":[[2018,11,7]],"date-time":"2018-11-07T03:45:22Z","timestamp":1541562322000},"page":"418","update-policy":"https:\/\/doi.org\/10.3390\/mdpi_crossmark_policy","source":"Crossref","is-referenced-by-count":29,"title":["Systemic Capillary Leak Syndrome (Clarkson Syndrome) in Cancer Patients: A Systematic Review"],"prefix":"10.3390","volume":"7","author":[{"ORCID":"https:\/\/orcid.org\/0000-0003-2326-1820","authenticated-orcid":false,"given":"Jae Il","family":"Shin","sequence":"first","affiliation":[{"name":"Department of Pediatrics, Yonsei University College of Medicine, Yonsei-ro 50, Seodaemun-gu, C.P.O. Box 8044, Seoul 03722, Korea"},{"name":"Department of Pediatric Nephrology, Severance Children\u2019s Hospital, Seoul 03722, Korea"},{"name":"Institute of Kidney Disease Research, Yonsei University College of Medicine, Seoul 03722, Korea"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-1511-9587","authenticated-orcid":false,"given":"Keum Hwa","family":"Lee","sequence":"additional","affiliation":[{"name":"Department of Pediatrics, Yonsei University College of Medicine, Yonsei-ro 50, Seodaemun-gu, C.P.O. Box 8044, Seoul 03722, Korea"},{"name":"Department of Pediatric Nephrology, Severance Children\u2019s Hospital, Seoul 03722, Korea"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"I. Re","family":"Lee","sequence":"additional","affiliation":[{"name":"Department of Pediatrics, Yonsei University College of Medicine, Yonsei-ro 50, Seodaemun-gu, C.P.O. Box 8044, Seoul 03722, Korea"},{"name":"Department of Pediatric Nephrology, Severance Children\u2019s Hospital, Seoul 03722, Korea"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ji Hyun","family":"Oh","sequence":"additional","affiliation":[{"name":"Wonkwang University School of Medicine, Iksan 54538, Korea"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Dong Wook","family":"Kim","sequence":"additional","affiliation":[{"name":"Department of Pediatrics, Yonsei University College of Medicine, Yonsei-ro 50, Seodaemun-gu, C.P.O. Box 8044, Seoul 03722, Korea"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jae Won","family":"Shin","sequence":"additional","affiliation":[{"name":"Department of Pediatrics, Yonsei University College of Medicine, Yonsei-ro 50, Seodaemun-gu, C.P.O. Box 8044, Seoul 03722, Korea"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Tae Seong","family":"Eo","sequence":"additional","affiliation":[{"name":"Department of Pediatrics, Yonsei University College of Medicine, Yonsei-ro 50, Seodaemun-gu, C.P.O. Box 8044, Seoul 03722, Korea"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-2945-2946","authenticated-orcid":false,"given":"Andreas","family":"Kronbichler","sequence":"additional","affiliation":[{"name":"Department of Internal Medicine IV (Nephrology and Hypertension), Medical University Innsbruck, 6020 Innsbruck, Austria"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Michael","family":"Eisenhut","sequence":"additional","affiliation":[{"name":"Luton &amp; Dunstable University Hospital NHS Foundation Trust, Lewsey Road, Luton LU4 ODZ, UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Hans J.","family":"Van der Vliet","sequence":"additional","affiliation":[{"name":"Department of Medical Oncology, Amsterdam UMC, Cancer Center Amsterdam, VU University, 1081 HV Amsterdam, The Netherlands"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"1968","published-online":{"date-parts":[[2018,11,6]]},"reference":[{"key":"ref_1","doi-asserted-by":"crossref","first-page":"193","DOI":"10.1016\/0002-9343(60)90018-8","article-title":"Cyclical edema and shock due to increased capillary permeability","volume":"29","author":"Clarkson","year":"1960","journal-title":"Am. 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