Engraftment Syndrome and Associated Cytokine Storm and Capillary Leak Syndrome

2017 ◽  
pp. 195-199
Author(s):  
Mala K. Talekar ◽  
Jason L. Freedman
Keyword(s):  
Capillary Leak Syndrome ◽  
Cytokine Storm ◽  
Capillary Leak ◽  
Engraftment Syndrome

scholarly journals New-Onset Systemic Capillary Leak Syndrome in an Adult Patient with COVID-19

2021 ◽  
Vol 2021 ◽  
pp. 1-3
Author(s):  
Daniel B. Knox ◽  
Vivian Lee ◽  
Lindsay Leither ◽  
Samuel M. Brown
Keyword(s):  
Adult Patient ◽  
Therapeutic Approach ◽  
Disease Process ◽  
Underlying Disease ◽  
Capillary Leak Syndrome ◽  
Cytokine Storm ◽  
Capillary Leak ◽  
Systemic Capillary Leak Syndrome ◽  
Laboratory Findings ◽  
New Onset

Systemic capillary leak syndrome (SCLS) is a rare disorder manifesting as shock, hemoconcentration, and hypoalbuminemia, which may be either idiopathic or secondary to an underlying disease process. We report a case of an adult with COVID-19 infection associated with new onset SCLS. Our case joins only two other cases of new SCLS associated with COVID-19 infection reported in the literature. The clinical and laboratory findings in this case are distinct from findings seen in COVID-19 cytokine storm syndrome. Whether our therapeutic approach was efficacious is unknown. Further research into causes and therapies for these syndromes is urgently indicated.

Probable Mechanisms Involved in Immunotoxin Mediated Capillary Leak Syndrome (CLS) and Recently Developed Countering Strategies

2018 ◽  
Vol 18 (5) ◽  
pp. 335-342
Author(s):  
B. Darvishi ◽  
L. Farahmand ◽  
N. Jalili ◽  
K. Majidzadeh-A
Keyword(s):  
Capillary Leak Syndrome ◽  
Capillary Leak

scholarly journals Continuous renal replacement therapy rescued life-threatening capillary leak syndrome in an extremely-low-birth-weight premature: a case report

2021 ◽  
Vol 47 (1) ◽  
Author(s):  
Li-Fen Yang ◽  
Jia-Chang Ding ◽  
Ling-Ping Zhu ◽  
Li-Xia Li ◽  
Meng-Qi Duan ◽  
...  
Keyword(s):  
Birth Weight ◽  
Renal Replacement Therapy ◽  
Low Birth Weight ◽  
Continuous Renal Replacement Therapy ◽  
Replacement Therapy ◽  
Therapeutic Option ◽  
Capillary Leak Syndrome ◽  
Extremely Low Birth Weight ◽  
Capillary Leak ◽  
Life Threatening

Abstract Background Capillary leak syndrome (CLS) is a rare disease characterized by profound vascular leakage and presents as a classic triad of hypotension, hypoalbuminemia and hemoconcentration. Severe CLS is mostly induced by sepsis and generally life-threatening in newborns, especially in premature infants. Continuous renal replacement therapy (CRRT) plays an important role of supportive treatment for severe CLS. Unfortunately, CRRT in preterm infants has rarely been well defined. Case presentation We report the case of a 11-day-old girl with CLS caused by sepsis, who was delivered by spontaneous vaginal delivery (SVD) at gestational age of 25 weeks and 4 days, and a birth weight of 0.89 Kilograms(kg). The infant received powerful management consisting of united antibiotics, mechanical ventilation, intravenous albumin and hydroxyethyl starch infusion, vasoactive agents, small doses of glucocorticoids and other supportive treatments. However, the condition rapidly worsened with systemic edema, hypotension, pulmonary exudation, hypoxemia and anuria in about 40 h. Finally, we made great efforts to perform CRRT for her. Fortunately, the condition improved after 82 h’ CRRT, and the newborn was rescued and gradually recovered. Conclusion CRRT is an effective rescue therapeutic option for severe CLS and can be successfully applied even in extremely-low-birth-weight premature.

scholarly journals Vascular endothelial hyperpermeability induces the clinical symptoms of Clarkson disease (the systemic capillary leak syndrome)

Blood ◽  
2012 ◽  
Vol 119 (18) ◽  
pp. 4321-4332 ◽  
Author(s):  
Zhihui Xie ◽  
Chandra C. Ghosh ◽  
Roshni Patel ◽  
Shoko Iwaki ◽  
Donna Gaskins ◽  
...  
Keyword(s):  
Clinical Symptoms ◽  
Case Series ◽  
Fiber Formation ◽  
Capillary Leak Syndrome ◽  
Actin Stress Fiber ◽  
Vascular Endothelial ◽  
Capillary Leak ◽  
Systemic Capillary Leak Syndrome ◽  
Humoral Factors ◽  
Clarkson Disease

Abstract The systemic capillary leak syndrome (SCLS) is a rare disorder characterized by transient episodes of hypotensive shock and anasarca thought to arise from reversible microvascular barrier dysfunction. Although the high prevalence of a monoclonal gammopathy of unknown significance in SCLS suggests a pathogenic contribution of endogenous immunoglobulins, the mechanisms of vascular hyperpermeability remain obscure. Herein, we report clinical and molecular findings on 23 patients, the largest SCLS case series to date. Application of episodic SCLS sera, but neither the purified immunoglobulin fraction nor sera obtained from patients during remission, to human microvascular endothelial cells caused vascular endothelial cadherin internalization, disruption of interendothelial junctions, actin stress fiber formation, and increased permeability in complementary functional assays without inducing endothelial apoptosis. Intravenous immunoglobulin, one promising therapy for SCLS, mitigated the permeability effects of episodic sera. Consistent with the presence of endogenous, nonimmunoglobulin, circulating permeability factor(s) constrained to SCLS episodes, we found that vascular endothelial growth factor (VEGF) and angiopoietin 2 (Ang2), were elevated in episodic SCLS sera but not in remission sera. Ab-based inhibition of Ang2 counteracted permeability induced by episodic SCLS sera. Comparable experiments with anti-VEGF Ab (bevacizumab) yielded less interpretable results, probably because of endothelial toxicity of VEGF withdrawal. Our results support a model of SCLS pathogenesis in which nonimmunoglobulin humoral factors such as VEGF and Ang2 contribute to transient endothelial contraction, suggesting a molecular mechanism for this highly lethal disorder.

Capillary-leak syndrome: an unrecognized early immune adverse effect of checkpoint-inhibitors treatment

Immunotherapy ◽  
2021 ◽  
Author(s):  
Ruth Percik ◽  
Asher Nethanel ◽  
Yair Liel
Keyword(s):  
Adverse Effect ◽  
Immune Response ◽  
Immune Checkpoint Inhibitors ◽  
Checkpoint Inhibitors ◽  
Endothelial Damage ◽  
Capillary Leak Syndrome ◽  
Capillary Leak ◽  
Cytokine Activation ◽  
Complex Interactions ◽  
Cellular Immune

Capillary-leak syndrome is strongly associated with cytokine activity states. It is an ill-recognized adverse effect of checkpoint inhibitors treatment, which are typically associated with cellular immune response. We describe two patients with capillary leak syndrome following immune checkpoint inhibitors treatment. We present linking mechanisms between checkpoint inhibitors, cellular immunity, cytokine action and endothelial damage. We suggest that capillary-leak syndrome is a unique adverse effect of immunotherapy, resulting from complex interactions between cellular and cytokine activation and that its expression is probably depending on inherent host immune variabilities.

Encephalopathy Due to Capillary Leak Syndrome

1997 ◽  
Vol 90 (10) ◽  
pp. 1060-1062 ◽  
Author(s):  
TULIO E. BERTORINI ◽  
MICHAEL S. GELFAND ◽  
THOMAS F. OʼBRIEN
Keyword(s):  
Capillary Leak Syndrome ◽  
Capillary Leak

scholarly journals A Remarkable Coexistence of Systemic Capillary Leak Syndrome and Diabetes in an 11-Year-Old Boy: A Case Report and Review of the Literature

2020 ◽  
Vol 2020 ◽  
pp. 1-5
Author(s):  
Aysun Ata ◽  
Samim Özen ◽  
Damla Gökşen ◽  
Neslihan Edeer Karaca ◽  
Güzide Aksu ◽  
...  
Keyword(s):  
Urine Analysis ◽  
Early Recognition ◽  
Recovery Period ◽  
Capillary Leak Syndrome ◽  
Ivig Treatment ◽  
Acid Decarboxylase ◽  
Diabetic Patients ◽  
Healthy Children ◽  
Capillary Leak ◽  
Systemic Capillary Leak Syndrome

Systemic capillary leak syndrome (ISCLS) is a rare disease characterized by unexplained reversible capillary hyperpermeability followed by hypoperfusion, hemoconcentration, and either hypoalbuminemia or total hypoproteinemia. An 11-year-old boy was admitted with vomiting, generalized edema, and hyperglycemia, which was preceded by 5 days of coryzal symptoms, lethargy, and oral aft, without fever. On physical examination, he had tachycardia and hypotension, with severe generalized systemic nonitchy edema, and the laboratory tests supported the conclusion that he had severe hemoconcentration with hemoglobin: 184 g/L, hematocrit: 51.3 %, urea: 20 mmol/L, blood glucose: 11.1 mmol/L, and albumin: 19 gr/L, with normal urine analysis. On the fourth day, the patient was diagnosed with ISCLS, by ruling out other causes of shock and hypoalbuminemia. Intravenous immunoglobulin (IVIG) treatment regimen was administered on two consecutive days (day five and day six). His edema decreased on the fifth day, and the patient was deemed clinically well. There was no compartment syndrome, rhabdomyolysis, or pulmonary edema in the recovery period. However, respiratory virus panel PCR was positive for respiratory syncytial virus (RSV) and enterovirus, which were thought to be the triggering cause of ISCLS. For the differential diagnosis of diabetes, his fasting serum glucose was 13.4 mmol/L, simultaneous C-peptide was 0.44 nmol/L, and HbA1c was 64 mmol/mol, and urine ketone was positive. However, antiglutamic acid decarboxylase, anti-insulin antibody, and islet cell antibody were negative. At the last outpatient visit, 22 months after the diagnosis, his insulin dose was still 0.4 IU/kg/day and HbA1c was 40 mmol/mol, and without prophylaxis, there was no ISCLS attack. Conclusion. Early recognition of ISCLS is important for therapeutic awareness, since it is very rare in childhood and occurs usually without any prior provoking factors in healthy children. With the increase in awareness of the disease, knowledge and experiences about pediatric patients may also increase. We think that our case will contribute to the literature since there have been no pediatric diabetic patients with ISCLS reported.

Masquerading as Septic Shock: Idiopathic Systemic Capillary Leak Syndrome

CHEST Journal ◽  
2014 ◽  
Vol 146 (4) ◽  
pp. 295A
Author(s):  
Kymberly McDonald ◽  
Vanessa Yap ◽  
Stephen Meng ◽  
Prashant Grover ◽  
Daniel Gerardi
Keyword(s):  
Septic Shock ◽  
Capillary Leak Syndrome ◽  
Capillary Leak ◽  
Systemic Capillary Leak Syndrome
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