The Korean Journal of Internal Medicine : 22:130-132, 2007 Two Cases of Systemic Capillary Leak Syndrome that were Treated with Pentastarch Chin Young Seok Lee, M.D., Sun Young Kim, M.D., Woo Kwon, M.D., Hae Geun Song, M.D., Young Kyung Lee, M.D., Ph.D.2, Hyo Jung Kim, M.D., Ph.D. and Dae Young Zang, M.D., Ph.D. Departments of Internal Medicine and Laboratory Medicine2, College of Medicine, Hallym University, Chuncheon, Korea Systemic capillary leak syndrome (SCLS) is a condition that's caused by the shift of fluid and protein from the intravascular space to the interstitial space as a result of repetitive episodes of capillary hyperpermeability. The pathogenesis of SCLS is still unclear, but there's recently been a report showing this syndrome in association with monoclonal gammopathy. This syndrome can be a fatal disease because cardiovascular collapse can occur in the initial capillary leak phase. Although theophylline, diuretics, terbutaline, steroids, calcium antagonist, Ginkgo biloba extracts and plasmapheresis have been suggested as medication, none of them have been proven to be effective. Considering that this disease is self-limiting, conservative treatment in the acute phase is believed to be very important. Because hypoalbuminemia is very a common manifestation of SCLS, Pentastarch, which has a higher molecular weight than albumin, could be efficient to prevent cardiovascular collapse. We used 10% Pentastarch during the acute SCLS attacks of 2 patients and the patients both showed a dramatic response. Pentastarch may be helpful to treat SCLS in its initial capillary leak phase by the elevating blood pressure, and this might contribute to somewhat decreasing the acute mortality of SCLS. Key Words : Systemic capillary leak syndrome, Pentastarch, Cardiovascular collapse INTRODUCTION Systemic capillary leak syndrome (SCLS) is a disorder characterized by unexplained episodic capillary hyperpermeability, which causes the shift of fluid and protein from the intravascular space to the interstitial space1). It is a rare, but often fatal disease1). Since no effective treatment has so far been found, this disease could lead to death if the blood pressure is not increased during the initial capillary leak phase. We experienced two cases where treatment with using 10% pentastarch elevated the blood pressure and eventually improved the patients, who were both in the acute phase of SCLS. CASE REPORT Case 1 A 37 year-old woman came to our hospital presenting with episodes of resting dyspnea, oliguria and anasarca, which had started one day previously. She had developed upper respiratory tract infection 3 days before admission, but she didn't get any medication. She was alert with a systolic blood pressure of 40 mmHg, heart rate: 110 beats/min, respiratory rate: 35 breaths/min and body temperature: 37℃. Auscultation proved her breath sounds were normal. The laboratory finding showed hemoglobin: 19.2 g/dL, hematocrit: 53%, leukocytes: 17,400/mm2, platelets: 147,000/mm2, blood urea nitrogen/ ∙Received : July 26, 2006 ∙Accepted : October 30, 2006 ∙Correspondence to : Hyo Jung Kim, M.D., Ph.D., Department of Internal Medicine, Hallym University Sacred Heart Hospital, Hallym University Medical Center, 896 Pyeongchon-dong, Dongan-gu, Anyang 431-070, Korea Tel : 82-31-380-3704, Fax : 82-31-386-2269 E-mail : hemonc@hallym.or.kr