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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

Vascular pathophysiology of sickle cell disease.
Connes P, Renoux C, Joly P, Nader E
Presse Med 2023 Dec;52(4):104202. Epub 2023 Nov 7 doi: 10.1016/j.lpm.2023.104202. PMID: 37944640
Sickle cell disease: an update.
Conway O'Brien E, Ali S, Chevassut T
Clin Med (Lond) 2022 May;22(3):218-220. doi: 10.7861/clinmed.2022-0143. PMID: 35584832Free PMC Article
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

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
Management of Osteomyelitis in Sickle Cell Disease: Review Article.
Al Farii H, Zhou S, Albers A
J Am Acad Orthop Surg Glob Res Rev 2020 Sep;4(9):e20.00002-10. doi: 10.5435/JAAOSGlobal-D-20-00002. PMID: 32890008Free 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

Apadamtase Alfa: First Approval.
Heo YA
Drugs 2024 Apr;84(4):467-472. doi: 10.1007/s40265-024-02007-6. PMID: 38418772
Managing pregnancy in patients with sickle cell disease from a transfusion perspective.
Habibi A, Benachi A, Lecarpentier E
Hematology Am Soc Hematol Educ Program 2023 Dec 8;2023(1):640-645. doi: 10.1182/hematology.2023000497. PMID: 38066847Free PMC Article
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

Increased Circulating Copeptin Levels Are Associated with Vaso-Occlusive Crisis and Right Ventricular Dysfunction in Sickle Cell Anemia.
Deveci OS, Ozmen C, Karaaslan MB, Celik AI, Rahimova H, Akray A, Tepe O, Gurkan E, Deniz A
Med Princ Pract 2022;31(1):47-53. Epub 2021 Dec 1 doi: 10.1159/000521216. PMID: 34852350Free 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 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
The cause and pathogenesis of hemolytic transfusion reactions in sickle-cell disease.
Pirenne F
Curr Opin Hematol 2019 Nov;26(6):488-494. doi: 10.1097/MOH.0000000000000546. PMID: 31589171

Clinical prediction guides

Pain Control for Sickle Cell Crisis, a Novel Approach? A Retrospective Study.
Rollé A, Vidal E, Laguette P, Garnier Y, Delta D, Martino F, Portecop P, Etienne-Julan M, Piednoir P, De Jong A, Romana M, Bernit E
Medicina (Kaunas) 2023 Dec 18;59(12) doi: 10.3390/medicina59122196. PMID: 38138299Free 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
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
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

Pain Control for Sickle Cell Crisis, a Novel Approach? A Retrospective Study.
Rollé A, Vidal E, Laguette P, Garnier Y, Delta D, Martino F, Portecop P, Etienne-Julan M, Piednoir P, De Jong A, Romana M, Bernit E
Medicina (Kaunas) 2023 Dec 18;59(12) doi: 10.3390/medicina59122196. PMID: 38138299Free 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
Micronutrients and sickle cell disease, effects on growth, infection and vaso-occlusive crisis: a systematic review.
Dekker LH, Fijnvandraat K, Brabin BJ, van Hensbroek MB
Pediatr Blood Cancer 2012 Aug;59(2):211-5. Epub 2012 Apr 10 doi: 10.1002/pbc.24163. PMID: 22492631

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