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Vaso-Occlusive Crisis

MedGen UID:
883122
•Concept ID:
C0750151
•
Disease or Syndrome
Synonym: Sickle Cell Crisis

Definition

An exacerbation of sickle cell disease. [from NCI]

Professional guidelines

PubMed

An evaluation of ticagrelor for the treatment of sickle cell anemia.
Ribeiro-Filho J, Yahouédéhou SCMA, Pitanga TN, Santana SS, Adorno EV, Barbosa CG, Ferreira JRD, Pina ETG, Neres JSDS, Leite IPR, Lyra IM, Goncalves MS
Expert Rev Hematol 2020 Oct;13(10):1047-1055. Epub 2020 Sep 24 doi: 10.1080/17474086.2020.1817736. PMID: 32972255
The vaso-occlusive pain crisis in sickle cell disease: Definition, pathophysiology, and management.
Darbari DS, Sheehan VA, Ballas SK
Eur J Haematol 2020 Sep;105(3):237-246. Epub 2020 May 19 doi: 10.1111/ejh.13430. PMID: 32301178
State of the Art Management of Acute Vaso-occlusive Pain in Sickle Cell Disease.
Puri L, Nottage KA, Hankins JS, Anghelescu DL
Paediatr Drugs 2018 Feb;20(1):29-42. doi: 10.1007/s40272-017-0263-z. PMID: 28853040

Recent clinical studies

Etiology

Lactate dehydrogenase and hemolysis index to predict vaso-occlusive crisis in sickle cell disease.
Feugray G, Dumesnil C, Grall M, Benhamou Y, Girot H, Fettig J, Brunel V, Billoir P
Sci Rep 2023 Dec 1;13(1):21198. doi: 10.1038/s41598-023-48324-w. PMID: 38040880Free PMC Article
A randomized clinical trial of the efficacy and safety of rivipansel for sickle cell vaso-occlusive crisis.
Dampier CD, Telen MJ, Wun T, Brown RC, Desai P, El Rassi F, Fuh B, Kanter J, Pastore Y, Rothman J, Taylor JG, Readett D, Sivamurthy KM, Tammara B, Tseng LJ, Lozier JN, Thackray H, Magnani JL, Hassell KL; RESET Investigators
Blood 2023 Jan 12;141(2):168-179. doi: 10.1182/blood.2022015797. PMID: 35981565
Voxelotor in adolescents and adults with sickle cell disease (HOPE): long-term follow-up results of an international, randomised, double-blind, placebo-controlled, phase 3 trial.
Howard J, Ataga KI, Brown RC, Achebe M, Nduba V, El-Beshlawy A, Hassab H, Agodoa I, Tonda M, Gray S, Lehrer-Graiwer J, Vichinsky E
Lancet Haematol 2021 May;8(5):e323-e333. Epub 2021 Apr 7 doi: 10.1016/S2352-3026(21)00059-4. PMID: 33838113
The vaso-occlusive pain crisis in sickle cell disease: Definition, pathophysiology, and management.
Darbari DS, Sheehan VA, Ballas SK
Eur J Haematol 2020 Sep;105(3):237-246. Epub 2020 May 19 doi: 10.1111/ejh.13430. PMID: 32301178
Crises in Sickle Cell Disease.
Novelli EM, Gladwin MT
Chest 2016 Apr;149(4):1082-93. Epub 2015 Dec 28 doi: 10.1016/j.chest.2015.12.016. PMID: 26836899Free PMC Article

Diagnosis

Lactate dehydrogenase and hemolysis index to predict vaso-occlusive crisis in sickle cell disease.
Feugray G, Dumesnil C, Grall M, Benhamou Y, Girot H, Fettig J, Brunel V, Billoir P
Sci Rep 2023 Dec 1;13(1):21198. doi: 10.1038/s41598-023-48324-w. PMID: 38040880Free PMC Article
Validation of patient-reported vaso-occlusive crisis day as an endpoint in sickle cell disease studies.
Coyne KS, Currie BM, Callaghan M, Wyrwich KW, Pease S, Baker CL, Arkin S, Pittman DD
Eur J Haematol 2022 Sep;109(3):226-237. Epub 2022 Jun 20 doi: 10.1111/ejh.13790. PMID: 35569114Free PMC Article
The vaso-occlusive pain crisis in sickle cell disease: Definition, pathophysiology, and management.
Darbari DS, Sheehan VA, Ballas SK
Eur J Haematol 2020 Sep;105(3):237-246. Epub 2020 May 19 doi: 10.1111/ejh.13430. PMID: 32301178
Rheumatic Manifestations of Haemoglobinopathies.
Hughes M
Curr Rheumatol Rep 2018 Aug 14;20(10):61. doi: 10.1007/s11926-018-0768-7. PMID: 30109514
Sickle Cell Disease in the Emergency Department.
Lovett PB, Sule HP, Lopez BL
Hematol Oncol Clin North Am 2017 Dec;31(6):1061-1079. doi: 10.1016/j.hoc.2017.08.009. PMID: 29078924

Therapy

A randomized clinical trial of the efficacy and safety of rivipansel for sickle cell vaso-occlusive crisis.
Dampier CD, Telen MJ, Wun T, Brown RC, Desai P, El Rassi F, Fuh B, Kanter J, Pastore Y, Rothman J, Taylor JG, Readett D, Sivamurthy KM, Tammara B, Tseng LJ, Lozier JN, Thackray H, Magnani JL, Hassell KL; RESET Investigators
Blood 2023 Jan 12;141(2):168-179. doi: 10.1182/blood.2022015797. PMID: 35981565
Antibiotics to modify sickle cell disease vaso-occlusive crisis?
Stewart C, Jang T, Mo G, Mohamed N, Poplawska M, Egini O, Dutta D, Lim SH
Blood Rev 2021 Nov;50:100867. Epub 2021 Jul 17 doi: 10.1016/j.blre.2021.100867. PMID: 34304939
Voxelotor in adolescents and adults with sickle cell disease (HOPE): long-term follow-up results of an international, randomised, double-blind, placebo-controlled, phase 3 trial.
Howard J, Ataga KI, Brown RC, Achebe M, Nduba V, El-Beshlawy A, Hassab H, Agodoa I, Tonda M, Gray S, Lehrer-Graiwer J, Vichinsky E
Lancet Haematol 2021 May;8(5):e323-e333. Epub 2021 Apr 7 doi: 10.1016/S2352-3026(21)00059-4. PMID: 33838113
The vaso-occlusive pain crisis in sickle cell disease: Definition, pathophysiology, and management.
Darbari DS, Sheehan VA, Ballas SK
Eur J Haematol 2020 Sep;105(3):237-246. Epub 2020 May 19 doi: 10.1111/ejh.13430. PMID: 32301178
Crises in Sickle Cell Disease.
Novelli EM, Gladwin MT
Chest 2016 Apr;149(4):1082-93. Epub 2015 Dec 28 doi: 10.1016/j.chest.2015.12.016. PMID: 26836899Free PMC Article

Prognosis

Lactate dehydrogenase and hemolysis index to predict vaso-occlusive crisis in sickle cell disease.
Feugray G, Dumesnil C, Grall M, Benhamou Y, Girot H, Fettig J, Brunel V, Billoir P
Sci Rep 2023 Dec 1;13(1):21198. doi: 10.1038/s41598-023-48324-w. PMID: 38040880Free PMC Article
Antibiotics to modify sickle cell disease vaso-occlusive crisis?
Stewart C, Jang T, Mo G, Mohamed N, Poplawska M, Egini O, Dutta D, Lim SH
Blood Rev 2021 Nov;50:100867. Epub 2021 Jul 17 doi: 10.1016/j.blre.2021.100867. PMID: 34304939
Voxelotor in adolescents and adults with sickle cell disease (HOPE): long-term follow-up results of an international, randomised, double-blind, placebo-controlled, phase 3 trial.
Howard J, Ataga KI, Brown RC, Achebe M, Nduba V, El-Beshlawy A, Hassab H, Agodoa I, Tonda M, Gray S, Lehrer-Graiwer J, Vichinsky E
Lancet Haematol 2021 May;8(5):e323-e333. Epub 2021 Apr 7 doi: 10.1016/S2352-3026(21)00059-4. PMID: 33838113
The impact of vaso-occlusive crises and disease severity on quality of life and productivity among patients with sickle cell disease in the US.
Shafrin J, Thom HHZ, Keeney E, Gaunt DM, Zhao LM, Bhor M, Rizio AA, Bronté-Hall L, Shah N
Curr Med Res Opin 2021 May;37(5):761-768. Epub 2021 Mar 27 doi: 10.1080/03007995.2021.1897556. PMID: 33686891
The vaso-occlusive pain crisis in sickle cell disease: Definition, pathophysiology, and management.
Darbari DS, Sheehan VA, Ballas SK
Eur J Haematol 2020 Sep;105(3):237-246. Epub 2020 May 19 doi: 10.1111/ejh.13430. PMID: 32301178

Clinical prediction guides

Lactate dehydrogenase and hemolysis index to predict vaso-occlusive crisis in sickle cell disease.
Feugray G, Dumesnil C, Grall M, Benhamou Y, Girot H, Fettig J, Brunel V, Billoir P
Sci Rep 2023 Dec 1;13(1):21198. doi: 10.1038/s41598-023-48324-w. PMID: 38040880Free PMC Article
Changes in Heme Levels During Acute Vaso-occlusive Crisis in Sickle Cell Anemia.
Cardoso EC, Silva-Neto PV, Hounkpe BW, Chenou F, Albuquerque CCMX, Garcia NP, Silva-Junior AL, Malheiro A, Cesar P, de Lima F, De Paula EV, Fraiji NA
Hematol Oncol Stem Cell Ther 2023 Jan 17;16(2):124-132. doi: 10.1016/j.hemonc.2021.08.002. PMID: 34450106
Antibiotics to modify sickle cell disease vaso-occlusive crisis?
Stewart C, Jang T, Mo G, Mohamed N, Poplawska M, Egini O, Dutta D, Lim SH
Blood Rev 2021 Nov;50:100867. Epub 2021 Jul 17 doi: 10.1016/j.blre.2021.100867. PMID: 34304939
Voxelotor in adolescents and adults with sickle cell disease (HOPE): long-term follow-up results of an international, randomised, double-blind, placebo-controlled, phase 3 trial.
Howard J, Ataga KI, Brown RC, Achebe M, Nduba V, El-Beshlawy A, Hassab H, Agodoa I, Tonda M, Gray S, Lehrer-Graiwer J, Vichinsky E
Lancet Haematol 2021 May;8(5):e323-e333. Epub 2021 Apr 7 doi: 10.1016/S2352-3026(21)00059-4. PMID: 33838113
Sickle red cell-endothelium interactions.
Kaul DK, Finnegan E, Barabino GA
Microcirculation 2009 Jan;16(1):97-111. doi: 10.1080/10739680802279394. PMID: 18720225Free PMC Article

Recent systematic reviews

Efficacy and safety of pharmacological interventions for managing sickle cell disease complications in children and adolescents: Systematic review with network meta-analysis.
Tonin FS, Ginete C, Ferreira J, Delgadinho M, Santos B, Fernandez-Llimos F, Brito M
Pediatr Blood Cancer 2023 Jun;70(6):e30294. Epub 2023 Mar 14 doi: 10.1002/pbc.30294. PMID: 36916826
Relevance of Plasma Homocysteine and Methylenetetrahydrofolate Reductase 677TT Genotype in Sickle Cell Disease: A Systematic Review and Meta-Analysis.
Ames PRJ, Arcaro A, Caruso M, Graf M, Marottoli V, Gentile F
Int J Mol Sci 2022 Nov 24;23(23) doi: 10.3390/ijms232314641. PMID: 36498990Free PMC Article
A systematic literature review of frequency of vaso-occlusive crises in sickle cell disease.
Zaidi AU, Glaros AK, Lee S, Wang T, Bhojwani R, Morris E, Donohue B, Paulose J, Iorga ŞR, Nellesen D
Orphanet J Rare Dis 2021 Nov 2;16(1):460. doi: 10.1186/s13023-021-02096-6. PMID: 34727959Free PMC Article
Manifestations of HbSE sickle cell disease: a systematic review.
Khamees I, Ata F, Choudry H, Soliman AT, De Sanctis V, Yassin MA
J Transl Med 2021 Jun 16;19(1):262. doi: 10.1186/s12967-021-02931-1. PMID: 34134694Free PMC Article
Magnesium for treating sickle cell disease.
Than NN, Soe HHK, Palaniappan SK, Abas AB, De Franceschi L
Cochrane Database Syst Rev 2017 Apr 14;4(4):CD011358. doi: 10.1002/14651858.CD011358.pub2. PMID: 28409830Free PMC Article

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