DOCSLIB.ORG

Kidney Dysfunction After Hematopoietic Cell Transplantation—Etiology, Management, and Perspectives

Total Page：16

File Type：pdf, Size：1020Kb

Download full-text PDF Read full-text
Kidney Dysfunction After Hematopoietic Cell Transplantation—Etiology, Management, and Perspectives Hematol Oncol Stem Cell Ther (2018) 11, 195– 205 Available at www.sciencedirect.com ScienceDirect journal homepage: www.elsevier.com/locate/hemonc REVIEW Kidney dysfunction after hematopoietic cell transplantation—Etiology, management, and perspectives Dorota Jagus´a, Karol Lis b, Longin Niemczyk a, Grzegorz W. Basak b,* a Department of Nephrology, Dialysis and Internal Medicine, Medical University of Warsaw, Warsaw, Poland b Department of Hematology, Oncology and Internal Medicine, Medical University of Warsaw, Warsaw, Poland Received 4 February 2018; received in revised form 26 April 2018; accepted 12 July 2018 Available online 29 July 2018 KEYWORDS Abstract Acute kidney injury; Kidney dysfunction is a common complication of hematopoietic cell transplantation (HCT) with Chronic kidney disease; proven negative impact on early and long-term mortality. Causes of this complication are Hematopoietic cell diverse, usually overlapping, and poorly understood. Therefore, management implicates multi- transplantation directional investigations and simultaneous treatment of suspected causes. The etiology is fre- quently unconfirmed due to a lack of specific markers and prevalence of contraindications to renal biopsy among HCT recipients. Herein, we provide a summary of etiology and propose an algorithm for evaluation of kidney injury after HCT. We also map out the most urgent areas for research that aim to identify patients at risk of severe renal injury and develop nephropro- tective strategies. Ó 2018 King Faisal Specialist Hospital & Research Centre. Published by Elsevier Ltd. This is an open access article under the CC BY-NC-ND license (http://creativecommons.org/licenses/by-nc- nd/4.0/). Contents Introduction . 196 Etiology . 197 Kidney injury before HCT . 197 Prerenal AKI. 197 * Corresponding author at: Department of Hematology, Oncology and Internal Medicine, Medical University of Warsaw, 1A Banacha Street, 02-097 Warsaw, Poland. E-mail address: [email protected] (G.W. Basak). https://doi.org/10.1016/j.hemonc.2018.07.004 1658-3876/Ó 2018 King Faisal Specialist Hospital & Research Centre. Published by Elsevier Ltd. This is an open access article under the CC BY-NC-ND license (http://creativecommons.org/licenses/by-nc-nd/4.0/). 196 D. Jagus´ et al. Conditioning. 197 TBI.................................................................... 197 Chemotherapy . 198 GvHD prophylaxis . 198 Veno-occlusive disease/sinusoidal obstruction syndrome. 199 GvHD.................................................................... 200 Transplant-associated microangiopathy . 200 Infective factors . 200 Sepsis . 200 BK virus and adenovirus . 200 Nephrotoxic drugs . 201 Management of kidney dysfunction after HCT . 201 Prevention of kidney injury . 202 Personalized drug dosing. 202 Nephroprotective agents . 202 Summary . 203 Conflict of interest . 203 References . 203 Introduction days, Shingai et al. [14] proved that development of AKI before engraftment is an indicator of significantly lower Annually, over 50,000 hematopoietic cell transplantations overall survival within 100 days after HCT, compared to (HCTs) are performed around the globe. According to the postengraftment AKI, at 56% versus 90%, respectively. European Society for Blood and Marrow Transplantation Diagnosis of AKI after HCT may be challenging. Serum (EBMT) statistics, both the number and life expectancy of concentration of creatinine depends on muscle mass, hydra- transplant recipients have been rising recently [1]. Even tion, and increases with latency: 1–3 days after injury of a though prevalence of acute kidney injury (AKI) and chronic nephron has occurred. Similarly, GFR estimation based on kidney disease (CKD) after HCT has been decreasing in the creatinine level is prone to errors. Comparisons of esti- recent years [2,3], the kidney injury remains a significant mated GFR and GFR measured via radioisotopic methods complication of HCT that affects the quality of life and mor- (involving iohexol, Tc-99m-diethylenetriaminepentaacetic tality of transplanted patients. Identification of risk factors, acid) showed significant differences among patients quali- understanding causes, early diagnosis, and treatment of kid- fied for HCT [15,16]. In consequence, under- and overesti- ney injury are important to develop safer transplantation mation of GFR carry a risk of toxic or inadequate dosage procedures. of chemotherapeutics, as well as delayed AKI diagnosis. AKI after HCT is usually defined as a doubling of base- Since radioisotopic methods are expensive and time con- line serum creatinine and/or decline in glomerular filtra- suming, new more sensitive and specific markers of kidney tion rate (GFR) of at least 50% within the first 100 days function are urgently needed. A useful tool for prediction after HCT. This stands for Stage 2 of AKI according to of AKI is HCT comorbidity index (HCT-CI). It was proven that Acute Kidney Injury Network (AKIN) and Kidney Disease: HCT-CI 1–2 is associated with a 2.4-fold higher risk of sev- Improving Global Outcomes (KDIGO) criteria or stadium ere AKI, while HCT-CI 3 increases the risk almost fivefold of Injury in Risk, Injury, Failure, Loss, End-Stage (RIFLE) [17]. 2 criteria, as it was proven that AKI of at least this stage CKD is defined as a decrease in GFR below 60 mL/min/m , is a negative predictor of long-term mortality [4]. Such which persists for at least 3 months. CKD was reported to defined AKI occurs in 12–21% of patients undergoing autol- occur in up to 4.5% of patients within 5 years post HCT, 7% ogous HCT (autoHCT) [4–6] and varies depending on the of whom progressed to end stage renal failure and required type of conditioning after allogeneic HCT (alloHCT): 36– RRT [18]. The frequency of CKD increases with time after 56% after myeloablative and 7–46% after reduced- HCT and differs substantially between allogeneic (14%) and intensity conditioning (RIC) [4,7–11]. The percentage of autologous transplantation recipients (4%) [18,19]. Identified patients with AKI that require renal replacement therapy risk factors include AKI in the early posttransplantation per- (RRT) is reported at a rate of 7%, 20–33%, and 4%, respec- iod, total body irradiation (TBI) as a conditioning regimen, tively [12]. However, AKI is variably defined in the HCT lit- presence of graft-versus-host disease (GvHD), and prolonged erature, which along with heterogenicity of analyzed use of calcineurin inhibitors (CNIs) [19,20]. Albuminuria groups, make comparisons between studies problematic. defined as urinary albumin to creatinine ratio (ACR, Independently from definitions and laboratory methods 30–300 mg/g) is a negative prognostic marker for survival, used to asses kidney function, AKI is correlated with progression of CKD, occurrence of GvHD, and bacteremia increased all cause mortality (ACM) and nonrelapse mortal- [21]. There is a linear correlation between the level of ACR ity (NRM) [4,7,9,10,13]. Interestingly, while the median and NRM during the first 100 days after HCT: mortality rises time of AKI occurrence varies between 20 days and 40 about 10% on every 100 mg/g ACR increase [21,22]. Kidney dysfunction after HCT—Etiology, management, and perspectives 197 Table 1 Etiology of kidney dysfunction after Hematopoietic Cell Transplantation (HCT) [23]. Prerenal Intrarenal Post-renal Decreased renal blood flow following Acute tubular necrosis (sepsis, Crystal nephropathy heavy fluid loss (vomiting, diarrhea, nephrotoxic drugs, contrast agents, Retroperitoneal fibrosis associated dehydration, mucosal damage, CRS, ES, VOD/SOS) with radiotherapy bleeding) Interstitial nephritis (induced by: Tumor infiltration of urinary tract Septic shock drugs, infections such as BK virus, Hemorrhagic cystitis CLS adenovirus, fungal infection) ES TAM VOD/SOS Complex origin: GvHD Tumor lysis syndrome Note. CLS = capillary leak syndrome; CRS = cytokine release syndrome; ES = engraftment syndrome; GvHD = graft versus host disease; SOS = sinusoidal obstructive syndrome; TAM = transplant-associated microangiopathy; VOD = veno-occlusive disease. Etiology Prerenal AKI Kidney injury after HCT most often has complex origins. Prerenal AKI may occur at any time during and after HCT. Table 1 shows possible causes of renal injury in relation to This may be a result of fluid loss caused by chemotherapy- its pathophysiology. The most important issues are dis- induced vomiting or diarrhea, iatrogenic fluid overload, cussed below. and tumor-lysis syndrome in cases when remission was not achieved before conditioning [15,27]. Kidney injury before HCT Two other complications that cause AKI in the early post- transplant period are capillary leak syndrome (CLS) and engraftment syndrome. Both are caused by release of proin- Patients qualified for HCT frequently have impaired kidney flammatory cytokines and may manifest after autoHCT and function prior to transplantation. This may be caused by alloHCT as fluid retention and noninfectious fever. The underlying diseases, comorbidities, or both. Glomeru- mechanism of kidney injury is prerenal—secondary to lonephritis develops in many hematological malignancies intravascular volume depletion and possibly also direct as a paraneoplastic syndrome and resolves with remission impact of inflammation on kidney tissue [28]. CLS often pre- of the underlying disease [23]. In multiple myeloma and sents within 2 weeks post HCT as peripheral edema and ser- other plasma-cell dyscrasias, accumulation of monoclonal osal effusion resistant to diuretics [29]. CLS as a component protein
Load more

Recommended publications
  • COVID-19 Vaccine Janssen: Contraindication in Individuals with Previous Capillary Leak Syndrome and Update on Thrombosis with Thrombocytopenia Syndrome
    Janssen Sciences Ireland UC Address: Airton Road, Tallaght, D24 WR89 Tel: +353 1 466 5200 Fax: +353 1 431 1058 www.janssen.ie 19the July 2021 COVID-19 Vaccine Janssen: Contraindication in individuals with previous capillary leak syndrome and update on thrombosis with thrombocytopenia syndrome Dear Healthcare Professional, Janssen-Cilag International NV in agreement with the European Medicines Agency and the Health Products Regulatory Authority (HPRA) would like to inform you of the following: Summary Capillary leak syndrome (CLS): • Very rare cases of capillary leak syndrome (CLS) have been reported in the first days after vaccination with COVID-19 Vaccine Janssen, in some cases with a fatal outcome. A history of CLS has been reported in at least one case. • COVID-19 Vaccine Janssen is now contraindicated in individuals who have previously experienced episodes of CLS. • CLS is characterised by acute episodes of oedema mainly affecting the limbs, hypotension, haemoconcentration and hypoalbuminaemia. Patients with an acute episode of CLS following vaccination require prompt recognition and treatment. Intensive supportive therapy is usually warranted. Thrombosis with thrombocytopenia syndrome (TTS): • Individuals diagnosed with thrombocytopenia within 3 weeks after vaccination with COVID-19 Vaccine Janssen should be actively investigated for signs of thrombosis. Similarly, individuals who present with thrombosis within 3 weeks of vaccination should be evaluated for thrombocytopenia. • TTS requires specialised clinical management. Healthcare professionals should consult applicable guidance and/ or consult specialists (e.g., haematologists, specialists in coagulation) to diagnose and treat this condition. Background on the safety concern COVID-19 Vaccine Janssen suspension for injection is indicated for active immunisation to prevent COVID-19 caused by SARS-CoV-2, in individuals 18 years of age and older.
    [Show full text]
  • Capillary Leak Syndrome
    The Medicine Forum Volume 17 Article 8 2016 Case Report: Uncontrolled Anasarca: Capillary Leak Syndrome Ankita Mehta, MD Thomas Jefferson University Hospital, [email protected] Mansi Shah, MD Jefferson Hospital Ambulatory Practice, Thomas Jefferson University Hospital, [email protected] Follow this and additional works at: https://jdc.jefferson.edu/tmf Part of the Internal Medicine Commons, and the Oncology Commons Let us know how access to this document benefits ouy Recommended Citation Mehta, MD, Ankita and Shah, MD, Mansi (2016) "Case Report: Uncontrolled Anasarca: Capillary Leak Syndrome," The Medicine Forum: Vol. 17 , Article 8. DOI: https://doi.org/10.29046/TMF.017.1.009 Available at: https://jdc.jefferson.edu/tmf/vol17/iss1/8 This Article is brought to you for free and open access by the Jefferson Digital Commons. The Jefferson Digital Commons is a service of Thomas Jefferson University's Center for Teaching and Learning (CTL). The Commons is a showcase for Jefferson books and journals, peer-reviewed scholarly publications, unique historical collections from the University archives, and teaching tools. The Jefferson Digital Commons allows researchers and interested readers anywhere in the world to learn about and keep up to date with Jefferson scholarship. This article has been accepted for inclusion in The Medicine Forum by an authorized administrator of the Jefferson Digital Commons. For more information, please contact: [email protected]. Mehta, MD and Shah, MD: Case Report: Uncontrolled Anasarca: Capillary Leak Syndrome ONCOLOGY Case Report: Uncontrolled Anasarca: Capillary Leak Syndrome Ankita Mehta, MD, and Mansi Shah, MD INTRODUCTION progression of her pancreatic cancer. She was ultimately discharged home with hospice care.
    [Show full text]
  • Common Terminology Criteria for Adverse Events
    Common Terminology Criteria for Adverse Events Version 3.0 Index Cancer Therapy Evaluation Program NATIONAL INSTITUTES OF HEALTH National Cancer Institute http://ctep.cancer.gov/ 10/22/2003 Public Health Service Cancer Therapy Evaluation Program National Institutes of Health National Cancer Institute Common Terminology Criteria for Adverse Events (CTCAE) - Index Terms Report Bethesda, Maryland 20892 -- A -- abdomen ENDOCRINE HEPATOBILIARY/PANCREAS Adrenal insufficiency 17 Liver dysfunction/failure (clinical) 34 GASTROINTESTINAL Pancreatitis 34 Colitis 19 PAIN Distension/bloating, abdominal 20 Pain --Select 55 Enteritis (inflammation of the small bowel) 21 VASCULAR Fistula, GI --Select 22 Portal vein flow 70 Typhlitis (cecal inflammation) 27 HEMORRHAGE/BLEEDING abducens Hemorrhage, GI --Select 31 See: CN VI HEPATOBILIARY/PANCREAS abnormal Pancreatitis 34 BLOOD/BONE MARROW INFECTION Myelodysplasia 4 Colitis, infectious (e.g., Clostridium difficile) 35 CONSTITUTIONAL SYMPTOMS Infection (documented clinically or microbiologically) with 35 Odor (patient odor) 11 Grade 3 or 4 neutrophils (ANC <1.0 x 10e9/L) --Select Infection with normal ANC or Grade 1 or 2 neutrophils -- 35 ENDOCRINE Select Neuroendocrine: 17 Infection with unknown ANC --Select 36 ADH secretion abnormality (e.g., SIADH or low ADH) Neuroendocrine: 17 MUSCULOSKELETAL/SOFT TISSUE gonadotropin secretion abnormality Soft tissue necrosis --Select 46 Neuroendocrine: 17 PAIN growth hormone secretion abnormality Neuroendocrine: 17 Pain --Select 55 prolactin hormone secretion abnormality
    [Show full text]
  • Life-Threatening Capillary Leak Syndrome
    on Tec ati hn nt o la lo p g s i e n s a r & Journal of Yi-Zhi et al., J Transplant Technol Res 2015, S4 T R f e o l s DOI: 10.4172/2161-0991.1000S4-003 a e a n ISSN:r 2161-0991 r c u h o J Transplantation Technologies & Research CaseResearch Report Article OpenOpen Access Access EditorialResearchCase Report Article OpenOpen Access Access Life-threatening Capillary Leak Syndrome in an Adult with Refractory Acute Myeloid Leukemia during Allogeneic Transplantation: a Case Report and Review of Literature Yi-Zhi J, Lai-Quan H, Gui-Ping S, Yan D, He-Sheng H and Dong-Ping H* Department of Hematology, The Affiliated Yijishan Hospital of Wannan Medical College, Wuhu, China Abstract Background: Although allogeneic hematopoietic stem cell transplantation (allo-HSCT) offers the possibility of cure for hematological malignancies, various complications have been described. Capillary leak syndrome (CLS) has been previously observed in HSCT patients. CLS is a rare disease characterized by recurrent episodes of generalized edema and severe hypotension along with hypoproteinemia. Case Report: A 27-year-old Chinese man, diagnosed with refractory acute myeloid leukemia, was treated with a haploidentical stem cell transplant combined with an unrelated umbilical cord blood unit. The patient developed fatal CLS during the 9th day of the conditioning therapy. Conclusion: Since it is difficult to distinguish between CLS and other early complications during allo-HSCT, our report highlights the need for rigorous investigation of identifying CLS and the increasing need of insightful diagnosis to manage any incidence of CLS.
    [Show full text]
  • Necrotizing Soft-Tissue Infections
    11/1/2020 Necrotizing Fasciitis ▪Is a surgical diagnosis characterized by friability of the superficial fascia, Necrotizing dishwater-gray exudate, and a notable Soft-Tissue absence of pus. Infections N ENGL J MED 2017;377:2253-65. 1 2 Necrotizing fasciitis type I: Necrotizing fasciitis type II ▪ Polymicrobial infection involving aerobic ▪ Often associated with gas in the tissue ▪ A monomicrobial infection. and anaerobic organisms. and thus is difficult to distinguish from gas gangrene ▪ Seen in the elderly or in those with ▪ Among gram-positive organisms, group A streptococcus is the underlying illnesses. ▪ Nonclostridial anaerobic cellulitis and most common pathogen, followed by MRSA. synergistic necrotizing cellulitis are type ▪ Predisposed I variants. Both occur in patients with ▪ May occur in any age group and in those without underlying illness ▪ Diabetic or decubitus ulcers diabetes and typically involve the feet, ▪ Hemorrhoids with rapid extension into the leg ▪ Aeromonas hydrophila (freshwater laceration) ▪ Rectal fissures ▪ Necrotizing fasciitis should be ▪ Vibrio vulnificus (saltwater laceration, ingestion of raw oysters, ▪ Episiotomies considered in patients with systemic cirrhosis) ▪ Colonic or urologic surgery or gynecologic manifestations of sepsis, such as procedures. tachycardia, leukocytosis, acidosis, or ▪ Necrotizing group A streptococcal and clostridial infections are marked hyperglycemia mediated by bacterial exotoxins and the host response ▪ Ludwigs- submandibular ▪ Lemierre’s –thrombophlebitis of jugular ▪ Fournier’s-urethral breach 3 4 Invasive Group A Streptococcal Soft-Tissue Infections Invasive Group A Streptococcal Soft-Tissue Infections (Streptococcus Continued pyogenes) ▪ Infection with a defined portal of bacterial entry: Infection that arises spontaneously in the deep tissue, without an overt wound or lesion: ▪ In 50% of patients with group A streptococcal necrotizing fasciitis or myonecrosis, are without a portal ▪ S.
    [Show full text]
  • Signal AR on Embolic and Thrombotic Events with COVID-19 Vaccine
    20th April 2021 EMA/268126/2021 Pharmacovigilance Risk Assessment Committee (PRAC) Signal assessment report on Embolic and Thrombotic events (SMQ) with COVID-19 Vaccine Janssen (Ad26.COV2-S [recombinant]) EPITT no:19689 Confirmation assessment report 3rd April 2021 Adoption of first PRAC Recommendation 9th April 2021 Submission of responses by MAH 15th April 2021 Preliminary assessment report 19th April 2021 Deadline for comments 19th April 2021 (8pm CET) Updated Rapporteur assessment 20th April 2021 (9am Adoption of 2nd PRAC recommendation 20th April 2021 Submission of responses by MAH 22nd April 2021 Rapporteur assessment 30th April 2021 Deadline for comments 30th April 2021 (8pm CET) Updated Rapporteur assessment report 3rd May 2021 Adoption of 3rd PRAC recommendation 06th May 2021 Official address Domenico Scarlattilaan 6 ● 1083 HS Amsterdam ● The Ne therla nds Address for visits and deliveries Refer to www.ema.europa.eu/how-to-fi nd-us An agency of the European Union Send us a question Go to www.ema.europa.eu/contact Telephone +31 (0)88 781 6000 © Euro pe an Me dici ne s Age ncy, 2021. Reproduction is authorised provided the source is acknowledged. Administrative information Active substance(s) (invented name) COVID-19 Vaccine (Ad26.COV2-S [recombinant]) – COVID-19 Vaccine Janssen suspension for injection (Other viral vaccines) Strength(s) All Pharmaceutical form(s) All Route(s) of administration All Indication(s) COVID-19 Vaccine Janssen is indicated for active immunisation to prevent COVID-19 caused by SARS-CoV-2 in individuals
    [Show full text]
  • Safety, Pharmacokinetics, Pharmacodynamics, and Antitumor Activity of Dalantercept, an Activin Receptor–Like Kinase-1 Ligand Trap, in Patients with Advanced Cancer
    Published OnlineFirst October 30, 2013; DOI: 10.1158/1078-0432.CCR-13-1840 Clinical Cancer Cancer Therapy: Clinical Research Safety, Pharmacokinetics, Pharmacodynamics, and Antitumor Activity of Dalantercept, an Activin Receptor–like Kinase-1 Ligand Trap, in Patients with Advanced Cancer Johanna C. Bendell1, Michael S. Gordon2, Herbert I. Hurwitz3, Suzanne F. Jones1, David S. Mendelson2, Gerard C. Blobe3, Neeraj Agarwal4, Carolyn H. Condon5, Dawn Wilson5, Amelia E. Pearsall5, Yijun Yang5, Ty McClure5, Kenneth M. Attie5, Matthew L. Sherman5, and Sunil Sharma4 Abstract Purpose: The angiogenesis inhibitor dalantercept (formerly ACE-041) is a soluble form of activin receptor–like kinase-1 (ALK1) that prevents activation of endogenous ALK1 by bone morphogenetic protein-9 (BMP9) and BMP10 and exhibits antitumor activity in preclinical models. This first-in-human study of dalantercept evaluated its safety, tolerability, pharmacokinetics, pharmacodynamics, and antitu- mor activity in adults with advanced solid tumors. Experimental Design: Patients in dose-escalating cohorts received dalantercept subcutaneously at one of seven dose levels (0.1–4.8 mg/kg) every 3 weeks until disease progression. Patients in an expansion cohort received dalantercept at 0.8 or 1.6 mg/kg every 3 weeks until disease progression. Results: In 37 patients receiving dalantercept, the most common treatment-related adverse events were peripheral edema, fatigue, and anemia. Edema and fluid retention were dose-limiting toxicities and responded to diuretic therapy. No clinically significant, treatment-related hypertension, proteinuria, gross hemorrhage, or gastrointestinal perforations were observed. One patient with refractory squamous cell cancer of the head and neck had a partial response, and 13 patients had stable disease according to RECISTv1.1, eight of whom had prolonged periods (12 weeks) of stable disease.
    [Show full text]
  • Veno-Occlusive Disease (VOD) AKA Hepatic Sinusoidal Obstruction Syndrome (SOS) Mira A
    Veno-Occlusive Disease (VOD) AKA Hepatic Sinusoidal Obstruction Syndrome (SOS) Mira A. Kohorst, MD MD Anderson Cancer Center Disclosures • No financial disclosures • No discussion of off-label use of medication Roadmap • Pathophysiology • Risk factors • Clinical presentation • Diagnosis • Treatment • Critical care management • Summary PATHOPHYSIOLOGY Injury to the hepatic venous endothelium (sinusoidal endothelial cells and zone 3 hepatocytes) – conditioning chemotherapy, gemtuzumab/inotuzumab, radiation (>30 Gy) Hepatocytes Space of Disse Sinusoid Sinusoidal endothelial cells Richardson PG et al. Expert Opin Drug Saf 2013;12:123–36 Activation of endothelial cells → ↑thrombosis, ↓fibrinolysis, ↑inflammation, ↓cytoskeletal structure → Narrowing of sinusoids Richardson PG et al. Expert Opin Drug Saf 2013;12:123–36 Increased thrombosis and decreased thrombolysis → VOD/SOS Richardson PG et al. Expert Opin Drug Saf 2013;12:123–36 RISK FACTORS Risk Factors Corbacioglu, Jabbour, and Mohty. Biology of Blood and Marrow Transplantation (2019) 25:1271-80. Risk Factors • Pediatric patients have a 2- to 3-fold higher incidence than adults, estimated at 20-30% – Infants 30% (as high as 60%) – Osteopetrosis 60% – Neuroblastoma 30% – Macrophage activating syndromes 30% – Thalassemia 30% Strouse et al. Biology of Blood and Marrow Transplantation (2018). Corbacioglu et al. Expert Rev Hematol (2012). Cesaro et al. Haematologica (2005). CLINICAL PRESENTATION Clinical Presentation Faraci et al. Biology of Blood and Marrow Transplantation. Oct 2018. Clinical Presentation • Weight gain (fluid retention) – first • Can see thrombocytopenia and platelet refractoriness • Hyperbilirubinemia/jaundice, transaminitis – Anicteric in ~30% of pediatric patients • Firm, painful hepatomegaly with ascites • Renal insufficiency (hepatorenal syndrome) in 50% – Need for dialysis in 25% • Multiorgan failure, hepatic encephalopathy, death Corbacioglu et al.
    [Show full text]
  • Systemic Capillary Leak Syndrome Associated with Hypovolemic Shock
    Saugel et al. Scandinavian Journal of Trauma, Resuscitation and Emergency Medicine 2010, 18:38 http://www.sjtrem.com/content/18/1/38 CASE REPORT Open Access SystemicCase report Capillary Leak Syndrome associated with hypovolemic shock and compartment syndrome. Use of transpulmonary thermodilution technique for volume management Bernd Saugel*1, Andreas Umgelter1, Friedrich Martin2, Veit Phillip1, Roland M Schmid1 and Wolfgang Huber1 Abstract Systemic Capillary Leak Syndrome (SCLS) is a rare disorder characterized by increased capillary hyperpermeability leading to hypovolemic shock due to a markedly increased shift of fluid and protein from the intravascular to the interstitial space. Hemoconcentration, hypoalbuminemia and a monoclonal gammopathy are characteristic laboratory findings. Here we present a patient who suffered from SCLS with hypovolemic shock and compartment syndrome of both lower legs and thighs. Volume and catecholamine management was guided using transpulmonary thermodilution. Extended hemodynamic monitoring for volume and catecholamine management as well as monitoring of muscle compartment pressure is of crucial importance in SCLS patients. Backround care unit (ICU) (hospital B) after emergency decompres- Systemic Capillary Leak Syndrome (SCLS) is a rare disor- sive fasciotomy in another hospital (hospital A) the previ- der characterized by unexplained, often recurrent, non ous day (fig. 1). sepsis-related episodes of increased capillary hyperper- On admission to hospital A the previous day the patient meability leading to hypovolemic shock due to a mark- had presented with severe muscle pain in the legs and a 2- edly increased shift of fluid and protein from the week history of flu-like illness and sore throat with fever intravascular to the interstitial space.
    [Show full text]
  • Cardiac Tamponade Caused by Pericardial Effusion in a Patient with Systemic Capillary Leak Syndrome
    Netherlands Journal of Critical Care Submitted July 2017; Accepted November 2017 CASE REPORT Cardiac tamponade caused by pericardial effusion in a patient with systemic capillary leak syndrome W.L. Zoetman1, J.E. Paramarta2, C. Bouman1, B.T. Sanou3, A.P.J. Vlaar1,2 Departments of 1Intensive Care Medicine, 2Internal Medicine and 3Anaesthesiology, Academic Medical Center, Amsterdam, the Netherlands Correspondence A.P.J. Vlaar - [email protected] Keywords - intensive care, critically ill, capillary leakage, shock, tamponade Abstract We present a patient with systemic capillary leak syndrome show any abnormalities, in particular no abnormal haemoglobin (SCLS), complicated by obstructive shock due to cardiac phenotyping. No treatment was initiated, follow-up of the tamponade. anaemia was performed in the outpatient clinic. Methods: SCLS is a rare disease characterised by hypotension, hypoalbuminaemia, haemoconcentration and generalised Table 1. Laboratory values illustrating the course of SCLS oedema. Several severe complications have been described, Laboratory test Out-patient ER ICU admis- ICU day 1 ICU day 2 clinic sion including acute kidney injury, rhabdomyolysis, compartment Haemoglobin (g/dL, 12.4 20.9 16.3 14.8 11.1 syndrome and death. We present a patient with SCLS, 13.7-16.9) complicated by rapidly progressive pericardial effusion with Haematocrit (L/L, 0.38 0.62 - - 0.32 cardiac tamponade, for which a lifesaving pericardiocentesis 0.35-0.4) was performed. Because SCLS is often confused with septic Creatinine (µmol/L, 52 118 172 175 169 shock and anaphylactic shock, an obstructive shock may be 65-95) easily missed. Albumin (g/L, 44 - <10 27 28 35-50) Although SCLS is a very rare disorder, it is important to be aware Creatinine kinase 264 149 44377 81622 of its existence and the possible life-threatening complications, (U/L) including cardiac tamponade, in order to improve the outcome for the patient.
    [Show full text]
  • Severe Cerebral Involvement Due to Idiopathic Systemic Capillary Leak Syndrome Ali Riza Gunes,€ Peter Berlit & Ralph Weber
    CASE REPORT Severe cerebral involvement due to idiopathic systemic capillary leak syndrome Ali Riza Gunes,€ Peter Berlit & Ralph Weber Department of Neurology, Alfried-Krupp-Hospital Essen, Alfried-Krupp-Str. 21, 45131 Essen, Germany Correspondence Key Clinical Message Ali Gunes,€ Department of Neurology, Alfried- Krupp-Hospital Essen, Alfried-Krupp-Str. 21, The idiopathic systemic capillary leak syndrome (ISCLS) is a rare disorder, 45131 Essen, Germany. characterized by recurrent attacks of hypotension, hypoalbuminemia, and Tel: +4920143441439; hemoconcentration, which is often misdiagnosed due to overlapping features Fax: +492014342377; with other diseases. Even though cerebral involvement is uncommon, a broad E-mail: [email protected] awareness is crucial, because of its life-threatening character. Funding Information Dr. Gunes€ received funding for trips from Keywords Boehringer Ingelheim for the Annual Meeting coma, critical care, neurology, systemic disease. 2014 of the German Neurological Society and from Bayer Vital for the MS-School Marburg 2014. Dr. Weber received honoraria for oral presentation from Boehringer Ingelheim, Bristol-Myers Squibb, Covidien, and from serving on a scientific advisory board of Covidien. Dr. Weber received funding for trips from Bayer Vital for the European Stroke Conference 2011 and Boehringer Ingelheim for the Annual Meeting 2012 of the German Neurological Society. Prof. Peter Berlit received honoraria for lecturing from Bayer, Biogen, CSL Behring, Desitin, Merck Serono, MSD, Novartis. Received: 9 September 2015; Revised: 25 December 2015; Accepted: 5 February 2016 doi: 10.1002/ccr3.525 Case the absence of albuminuria, hemoconcentration (hemat- ocrit 55%), and a known IgG monoclonal gammopathy. We report the case of a 43-year-old woman who was The initial MR of the brain showed contrast agent referred to our hospital with coma of unknown origin.
    [Show full text]
  • Systemic Capillary Leak Syndrome Successfully Treated with a TNF-A
    C al & ellu ic la n r li Im C m f u Journal of o n l o a Suyama et al., J Clin Cell Immunol 2014, 5:5 l n o r g u y o J DOI: 10.4172/2155-9899.1000266 ISSN: 2155-9899 Clinical & Cellular Immunology Letter to Editor Open Access Systemic Capillary Leak Syndrome Successfully Treated with a TNF-α Inhibitor Yasuhiro Suyama1*, Mitsumasa Kishimoto1, Ryo Rokutanda1, Chisum Min1, Gautam A Deshpande2, Youichiro Haji1, Ken-ichi Yamaguchi1, Akira Takeda1, Yukio Matsui1 and Masato Okada1 1Immuno-Rheumatology Center, St. Luke’s International Hospital, St. Luke’s International University, 9-1 Akashi-cho Chuo-Ku, Tokyo 104-8560, Japan 2Center for Clinical Epidemiology, St Luke’s Life Science Institute, 10-1 Akashi-cho Chuo-Ku, Tokyo 104-0044, Japan *Corresponding author: Yasuhiro Suyama, Immuno-Rheumatology Center, St. Luke's International Hospital, St. Luke’s International University, 9-1 Akashi-cho, Chuo-ku, Tokyo 104-8560, Japan, Tel: +81-3-3541-5151; Fax: +81-3-5550-6000; E-mail: [email protected] Received date: August 29, 2014, Accepted date: October 23, 2014, Published date: October 30, 2014 Copyright: © 2014 Suyama Y, et al. This is an open-access article distributed under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited. Letter blood pressure was observed. On the 22nd day of admission, he was discharged home without any sequellae. We read with interest the excellent article by Zhihui Xie et al., reporting in 35 patients with acute systemic capillary leak syndrome This is the first report of successful treatment of an adult case of (SCLS) the elevation of several cytokines, including TNFα, compared SCLS with the TNF-α inhibitor, infliximab.
    [Show full text]