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Capillary leak syndrome

MedGen UID:
137987
•Concept ID:
C0343084
•
Disease or Syndrome
Synonym: Systemic capillary leak syndrome
SNOMED CT: Capillary leak syndrome (87730004)
Modes of inheritance:
Not genetically inherited
MedGen UID:
988794
•Concept ID:
CN307044
•
Finding
Source: Orphanet
clinical entity without genetic inheritance.
See: This record
Not genetically inherited (Orphanet)
 
Monarch Initiative: MONDO:0001956
Orphanet: ORPHA188

Definition

Systemic capillary leak syndrome (SCLS) is a severe systemic disease due to increased capillary permeability, characterized by episodes of hypotension, edema and hypovolemia. [from ORDO]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • Capillary leak syndrome
Follow this link to review classifications for Capillary leak syndrome in Orphanet.

Professional guidelines

PubMed

Transplant-associated thrombotic microangiopathy: Diagnostic challenges and management strategies.
Elemary M, Sabry W, Seghatchian J, Goubran H
Transfus Apher Sci 2019 Jun;58(3):347-350. Epub 2019 May 1 doi: 10.1016/j.transci.2019.04.022. PMID: 31097308
Recognition and management of idiopathic systemic capillary leak syndrome: an evidence-based review.
Baloch NU, Bikak M, Rehman A, Rahman O
Expert Rev Cardiovasc Ther 2018 May;16(5):331-340. Epub 2018 Apr 2 doi: 10.1080/14779072.2018.1456920. PMID: 29564922
Capillary leak syndrome: etiologies, pathophysiology, and management.
Siddall E, Khatri M, Radhakrishnan J
Kidney Int 2017 Jul;92(1):37-46. Epub 2017 Mar 17 doi: 10.1016/j.kint.2016.11.029. PMID: 28318633

Recent clinical studies

Etiology

Long-Term Benefits of Tagraxofusp for Patients With Blastic Plasmacytoid Dendritic Cell Neoplasm.
Pemmaraju N, Sweet KL, Stein AS, Wang ES, Rizzieri DA, Vasu S, Rosenblat TL, Brooks CL, Habboubi N, Mughal TI, Kantarjian H, Konopleva M, Lane AA
J Clin Oncol 2022 Sep 10;40(26):3032-3036. Epub 2022 Jul 12 doi: 10.1200/JCO.22.00034. PMID: 35820082Free PMC Article
Fatal Exacerbations of Systemic Capillary Leak Syndrome Complicating Coronavirus Disease.
Cheung PC, Eisch AR, Maleque N, Polly DM, Auld SC, Druey KM
Emerg Infect Dis 2021 Oct;27(10):2529-2534. Epub 2021 Jul 27 doi: 10.3201/eid2710.211155. PMID: 34314669Free PMC Article
Capillary leak syndrome: etiologies, pathophysiology, and management.
Siddall E, Khatri M, Radhakrishnan J
Kidney Int 2017 Jul;92(1):37-46. Epub 2017 Mar 17 doi: 10.1016/j.kint.2016.11.029. PMID: 28318633
Systemic capillary leak syndrome and autoimmune diseases: A case series.
Guffroy A, Dervieux B, Gravier S, Martinez C, Deibener-Kaminsky J, Hachulla E, Michel M, Weber JC, Korganow AS, Arnaud L, Gottenberg JE, Sibilia J; Club des Rhumatismes et Inflammations (CRI)
Semin Arthritis Rheum 2017 Feb;46(4):509-512. Epub 2016 Aug 12 doi: 10.1016/j.semarthrit.2016.08.001. PMID: 27637319
Anti-GD2 antibody with GM-CSF, interleukin-2, and isotretinoin for neuroblastoma.
Yu AL, Gilman AL, Ozkaynak MF, London WB, Kreissman SG, Chen HX, Smith M, Anderson B, Villablanca JG, Matthay KK, Shimada H, Grupp SA, Seeger R, Reynolds CP, Buxton A, Reisfeld RA, Gillies SD, Cohn SL, Maris JM, Sondel PM; Children's Oncology Group
N Engl J Med 2010 Sep 30;363(14):1324-34. doi: 10.1056/NEJMoa0911123. PMID: 20879881Free PMC Article

Diagnosis

Blastic Plasmacytoid Dendritic Cell Neoplasm.
Jain A, Sweet K
J Natl Compr Canc Netw 2023 May;21(5):515-521. doi: 10.6004/jnccn.2023.7026. PMID: 37156483
Capillary leak syndrome: State of the art in 2021.
Bichon A, Bourenne J, Gainnier M, Carvelli J
Rev Med Interne 2021 Nov;42(11):789-796. Epub 2021 Jun 4 doi: 10.1016/j.revmed.2021.05.012. PMID: 34099313
Capillary leak syndrome: etiologies, pathophysiology, and management.
Siddall E, Khatri M, Radhakrishnan J
Kidney Int 2017 Jul;92(1):37-46. Epub 2017 Mar 17 doi: 10.1016/j.kint.2016.11.029. PMID: 28318633
Idiopathic systemic capillary leak syndrome (Clarkson disease).
Druey KM, Parikh SM
J Allergy Clin Immunol 2017 Sep;140(3):663-670. Epub 2016 Dec 22 doi: 10.1016/j.jaci.2016.10.042. PMID: 28012935Free PMC Article
Erythroderma.
Rothe MJ, Bialy TL, Grant-Kels JM
Dermatol Clin 2000 Jul;18(3):405-15. doi: 10.1016/s0733-8635(05)70189-3. PMID: 10943536

Therapy

Blastic Plasmacytoid Dendritic Cell Neoplasm.
Jain A, Sweet K
J Natl Compr Canc Netw 2023 May;21(5):515-521. doi: 10.6004/jnccn.2023.7026. PMID: 37156483
Cytokine release syndrome and neurotoxicity following CAR T-cell therapy for hematologic malignancies.
Freyer CW, Porter DL
J Allergy Clin Immunol 2020 Nov;146(5):940-948. Epub 2020 Aug 6 doi: 10.1016/j.jaci.2020.07.025. PMID: 32771558
Tagraxofusp in Blastic Plasmacytoid Dendritic-Cell Neoplasm.
Pemmaraju N, Lane AA, Sweet KL, Stein AS, Vasu S, Blum W, Rizzieri DA, Wang ES, Duvic M, Sloan JM, Spence S, Shemesh S, Brooks CL, Balser J, Bergstein I, Lancet JE, Kantarjian HM, Konopleva M
N Engl J Med 2019 Apr 25;380(17):1628-1637. doi: 10.1056/NEJMoa1815105. PMID: 31018069
Capillary leak syndrome: etiologies, pathophysiology, and management.
Siddall E, Khatri M, Radhakrishnan J
Kidney Int 2017 Jul;92(1):37-46. Epub 2017 Mar 17 doi: 10.1016/j.kint.2016.11.029. PMID: 28318633
Anti-GD2 antibody with GM-CSF, interleukin-2, and isotretinoin for neuroblastoma.
Yu AL, Gilman AL, Ozkaynak MF, London WB, Kreissman SG, Chen HX, Smith M, Anderson B, Villablanca JG, Matthay KK, Shimada H, Grupp SA, Seeger R, Reynolds CP, Buxton A, Reisfeld RA, Gillies SD, Cohn SL, Maris JM, Sondel PM; Children's Oncology Group
N Engl J Med 2010 Sep 30;363(14):1324-34. doi: 10.1056/NEJMoa0911123. PMID: 20879881Free PMC Article

Prognosis

Blastic Plasmacytoid Dendritic Cell Neoplasm.
Jain A, Sweet K
J Natl Compr Canc Netw 2023 May;21(5):515-521. doi: 10.6004/jnccn.2023.7026. PMID: 37156483
Long-Term Benefits of Tagraxofusp for Patients With Blastic Plasmacytoid Dendritic Cell Neoplasm.
Pemmaraju N, Sweet KL, Stein AS, Wang ES, Rizzieri DA, Vasu S, Rosenblat TL, Brooks CL, Habboubi N, Mughal TI, Kantarjian H, Konopleva M, Lane AA
J Clin Oncol 2022 Sep 10;40(26):3032-3036. Epub 2022 Jul 12 doi: 10.1200/JCO.22.00034. PMID: 35820082Free PMC Article
Capillary leak syndrome: etiologies, pathophysiology, and management.
Siddall E, Khatri M, Radhakrishnan J
Kidney Int 2017 Jul;92(1):37-46. Epub 2017 Mar 17 doi: 10.1016/j.kint.2016.11.029. PMID: 28318633
Idiopathic systemic capillary leak syndrome (Clarkson disease).
Druey KM, Parikh SM
J Allergy Clin Immunol 2017 Sep;140(3):663-670. Epub 2016 Dec 22 doi: 10.1016/j.jaci.2016.10.042. PMID: 28012935Free PMC Article
Erythroderma.
Rothe MJ, Bialy TL, Grant-Kels JM
Dermatol Clin 2000 Jul;18(3):405-15. doi: 10.1016/s0733-8635(05)70189-3. PMID: 10943536

Clinical prediction guides

Long-Term Benefits of Tagraxofusp for Patients With Blastic Plasmacytoid Dendritic Cell Neoplasm.
Pemmaraju N, Sweet KL, Stein AS, Wang ES, Rizzieri DA, Vasu S, Rosenblat TL, Brooks CL, Habboubi N, Mughal TI, Kantarjian H, Konopleva M, Lane AA
J Clin Oncol 2022 Sep 10;40(26):3032-3036. Epub 2022 Jul 12 doi: 10.1200/JCO.22.00034. PMID: 35820082Free PMC Article
Mesothelin-Targeted Recombinant Immunotoxins for Solid Tumors.
Hagerty BL, Pegna GJ, Xu J, Tai CH, Alewine C
Biomolecules 2020 Jun 28;10(7) doi: 10.3390/biom10070973. PMID: 32605175Free PMC Article
Capillary leak syndrome: etiologies, pathophysiology, and management.
Siddall E, Khatri M, Radhakrishnan J
Kidney Int 2017 Jul;92(1):37-46. Epub 2017 Mar 17 doi: 10.1016/j.kint.2016.11.029. PMID: 28318633
Anti-GD2 antibody with GM-CSF, interleukin-2, and isotretinoin for neuroblastoma.
Yu AL, Gilman AL, Ozkaynak MF, London WB, Kreissman SG, Chen HX, Smith M, Anderson B, Villablanca JG, Matthay KK, Shimada H, Grupp SA, Seeger R, Reynolds CP, Buxton A, Reisfeld RA, Gillies SD, Cohn SL, Maris JM, Sondel PM; Children's Oncology Group
N Engl J Med 2010 Sep 30;363(14):1324-34. doi: 10.1056/NEJMoa0911123. PMID: 20879881Free PMC Article
Hantavirus infections.
Escutenaire S, Pastoret PP
Rev Sci Tech 2000 Apr;19(1):64-78. doi: 10.20506/rst.19.1.1209. PMID: 11189727

Recent systematic reviews

Immune checkpoint inhibitor-associated capillary leak syndrome: A systematic review and a worldwide pharmacovigilance study.
Wong So J, Bouibede F, Jonville-Béra AP, Maillot F, Barbier F, Largeau B
J Intern Med 2023 Jul;294(1):58-68. Epub 2023 Apr 20 doi: 10.1111/joim.13641. PMID: 37038359
Engraftment Syndrome and Acute Graft-versus-Host Disease: A Meta-Analysis.
Poonsombudlert K, Kewcharoen J, Prueksapraopong C, Limpruttidham N
Hawaii J Health Soc Welf 2020 Jun 1;79(6):194-201. PMID: 32524098Free PMC Article
Idiopathic systemic capillary leak syndrome (Clarkson syndrome) in childhood: systematic literature review.
Bozzini MA, Milani GP, Bianchetti MG, Fossali EF, Lava SAG
Eur J Pediatr 2018 Aug;177(8):1149-1154. Epub 2018 Jun 23 doi: 10.1007/s00431-018-3189-8. PMID: 29936591
Clinical Presentation, Management, and Prognostic Factors of Idiopathic Systemic Capillary Leak Syndrome: A Systematic Review.
Eo TS, Chun KJ, Hong SJ, Kim JY, Lee IR, Lee KH, Eisenhut M, Kronbichler A, Shin JI
J Allergy Clin Immunol Pract 2018 Mar-Apr;6(2):609-618. Epub 2017 Sep 19 doi: 10.1016/j.jaip.2017.07.021. PMID: 28939140
Acute hypopituitarism complicating Russell's viper envenomation: case series and systematic review.
Rajagopala S, Thabah MM, Ariga KK, Gopalakrishnan M
QJM 2015 Sep;108(9):719-28. Epub 2015 Jan 27 doi: 10.1093/qjmed/hcv011. PMID: 25630907

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