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Capillary leak

MedGen UID:
730470
Concept ID:
C1382398
Finding
Synonyms: Increased capillary permeability; Systemic capillary leak syndrome
SNOMED CT: Increased capillary permeability (1608005)
 
HPO: HP:0030005

Definition

An acute phenomenon characterized by hypotension and anasarca due to the loss of plasma volume into peripheral tissues, with evidence of decreased plasma volume (hemoconcentration) and protein loss from the intravascular space (hypoalbuminemia) during acute episodes. [from HPO]

Term Hierarchy

Conditions with this feature

Severe combined immunodeficiency due to LCK deficiency
MedGen UID:
862670
Concept ID:
C4014233
Disease or Syndrome
Immunodeficiency-22 (IMD22) is an autosomal recessive disorder characterized by the onset of recurrent bacterial, viral, and fungal respiratory, gastrointestinal, and skin infections in infancy or early childhood. Immunologic workup shows severe T-cell lymphopenia, particularly affecting the CD4+ subset, and impaired proximal TCR intracellular signaling and activation. Although NK cells and B cells are normal, some patients may have hypogammaglobulinemia secondary to the T-cell defect. There are variable manifestations, likely due to the severity of the particular LCK mutation: patients may develop prominent skin lesions resembling epidermodysplasia verruciformis, gastrointestinal inflammation, and virus-induced malignancy. The disease can be fatal in childhood, but hematopoietic stem cell transplant (HSCT) may be curative (Hauck et al., 2012; Li et al., 2016; Keller et al., 2023).

Professional guidelines

PubMed

Argaiz ER, Rola P, Haycock KH, Verbrugge FH
Eur Heart J Acute Cardiovasc Care 2022 Nov 2;11(10):786-793. doi: 10.1093/ehjacc/zuac104. PMID: 36069621
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
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

Argaiz ER, Rola P, Haycock KH, Verbrugge FH
Eur Heart J Acute Cardiovasc Care 2022 Nov 2;11(10):786-793. doi: 10.1093/ehjacc/zuac104. PMID: 36069621
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
Hay KA, Hanafi LA, Li D, Gust J, Liles WC, Wurfel MM, López JA, Chen J, Chung D, Harju-Baker S, Cherian S, Chen X, Riddell SR, Maloney DG, Turtle CJ
Blood 2017 Nov 23;130(21):2295-2306. Epub 2017 Sep 18 doi: 10.1182/blood-2017-06-793141. PMID: 28924019Free PMC Article
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
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

Jain A, Sweet K
J Natl Compr Canc Netw 2023 May;21(5):515-521. doi: 10.6004/jnccn.2023.7026. PMID: 37156483
Mudhol RR, Bhise R
J Assoc Physicians India 2022 Oct;70(10):11-12. doi: 10.5005/japi-11001-0106. PMID: 37355874
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
De Feo G, Parente R, Triggiani M
Curr Opin Allergy Clin Immunol 2018 Oct;18(5):382-386. doi: 10.1097/ACI.0000000000000468. PMID: 30028728
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

Therapy

Jain A, Sweet K
J Natl Compr Canc Netw 2023 May;21(5):515-521. doi: 10.6004/jnccn.2023.7026. PMID: 37156483
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
Frey N, Porter D
Biol Blood Marrow Transplant 2019 Apr;25(4):e123-e127. Epub 2018 Dec 23 doi: 10.1016/j.bbmt.2018.12.756. PMID: 30586620
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
Malbrain ML, Marik PE, Witters I, Cordemans C, Kirkpatrick AW, Roberts DJ, Van Regenmortel N
Anaesthesiol Intensive Ther 2014 Nov-Dec;46(5):361-80. doi: 10.5603/AIT.2014.0060. PMID: 25432556

Prognosis

Jain A, Sweet K
J Natl Compr Canc Netw 2023 May;21(5):515-521. doi: 10.6004/jnccn.2023.7026. PMID: 37156483
Thomas KE, Fotaki A, Botnar RM, Ferreira VM
Circ Cardiovasc Imaging 2023 Jan;16(1):e014068. Epub 2023 Jan 17 doi: 10.1161/CIRCIMAGING.122.014068. PMID: 36649450Free PMC Article
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
Hay KA, Hanafi LA, Li D, Gust J, Liles WC, Wurfel MM, López JA, Chen J, Chung D, Harju-Baker S, Cherian S, Chen X, Riddell SR, Maloney DG, Turtle CJ
Blood 2017 Nov 23;130(21):2295-2306. Epub 2017 Sep 18 doi: 10.1182/blood-2017-06-793141. PMID: 28924019Free PMC Article
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

Clinical prediction guides

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
Hay KA, Hanafi LA, Li D, Gust J, Liles WC, Wurfel MM, López JA, Chen J, Chung D, Harju-Baker S, Cherian S, Chen X, Riddell SR, Maloney DG, Turtle CJ
Blood 2017 Nov 23;130(21):2295-2306. Epub 2017 Sep 18 doi: 10.1182/blood-2017-06-793141. PMID: 28924019Free PMC Article
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
Malbrain ML, Marik PE, Witters I, Cordemans C, Kirkpatrick AW, Roberts DJ, Van Regenmortel N
Anaesthesiol Intensive Ther 2014 Nov-Dec;46(5):361-80. doi: 10.5603/AIT.2014.0060. PMID: 25432556
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

Recent systematic reviews

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
Beber A, Dellai F, Abdel Jaber M, Peterlana D, Brunori G, Maino A
Scand J Rheumatol 2022 Jan;51(1):67-69. Epub 2021 Jun 25 doi: 10.1080/03009742.2021.1917145. PMID: 34169783
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
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
Malbrain ML, Marik PE, Witters I, Cordemans C, Kirkpatrick AW, Roberts DJ, Van Regenmortel N
Anaesthesiol Intensive Ther 2014 Nov-Dec;46(5):361-80. doi: 10.5603/AIT.2014.0060. PMID: 25432556

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