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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

TH1/TH2 repolarization in induction of immune tolerance to non-steroidal anti-inflammatory drugs during the management of sickle cell disease vaso-occlusive crisis.
Romualde DS, Liliane SK, Koffi N, Honoré AA, Richard YO, Aniela AAU, Patricia-Victorine KA, Jocelyne SY, Roselle MLC, Salimata M, Doris O
Egypt J Immunol 2023 Oct;30(4):67-76. PMID: 37794629
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

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
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
Immunological Hallmarks of Inflammatory Status in Vaso-Occlusive Crisis of Sickle Cell Anemia Patients.
Silva-Junior AL, Garcia NP, Cardoso EC, Dias S, Tarragô AM, Fraiji NA, Gomes MS, Amaral LR, Teixeira-Carvalho A, Martins-Filho OA, De Paula EV, Costa AG, Malheiro A
Front Immunol 2021;12:559925. Epub 2021 Mar 11 doi: 10.3389/fimmu.2021.559925. PMID: 33776989Free 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
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
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
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
Current challenges in the management of patients with sickle cell disease - A report of the Italian experience.
Russo G, De Franceschi L, Colombatti R, Rigano P, Perrotta S, Voi V, Palazzi G, Fidone C, Quota A, Graziadei G, Pietrangelo A, Pinto V, Ruffo GB, Sorrentino F, Venturelli D, Casale M, Ferrara F, Sainati L, Cappellini MD, Piga A, Maggio A, Forni GL
Orphanet J Rare Dis 2019 May 30;14(1):120. doi: 10.1186/s13023-019-1099-0. PMID: 31146777Free PMC Article
Perioperative considerations for patients with sickle cell disease: a narrative review.
Khurmi N, Gorlin A, Misra L
Can J Anaesth 2017 Aug;64(8):860-869. Epub 2017 Apr 28 doi: 10.1007/s12630-017-0883-3. PMID: 28455727

Therapy

L-glutamine for sickle cell disease: more than reducing redox.
Jafri F, Seong G, Jang T, Cimpeanu E, Poplawska M, Dutta D, Lim SH
Ann Hematol 2022 Aug;101(8):1645-1654. Epub 2022 May 14 doi: 10.1007/s00277-022-04867-y. PMID: 35568758
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
Biochemical and therapeutic effects of Omega-3 fatty acids in sickle cell disease.
Daak AA, Lopez-Toledano MA, Heeney MM
Complement Ther Med 2020 Aug;52:102482. Epub 2020 Jun 9 doi: 10.1016/j.ctim.2020.102482. PMID: 32951732
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
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
Serum copeptin and cortisol do not accurately predict sickle cell anaemia vaso-occlusive crisis as C-reactive protein.
Akinlade KS, Atere AD, Olaniyi JA, Rahamon SK, Adewale CO
PLoS One 2013;8(11):e77913. Epub 2013 Nov 4 doi: 10.1371/journal.pone.0077913. PMID: 24223742Free PMC Article

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
Acute kidney injury in hospitalized children with sickle cell anemia.
Batte A, Menon S, Ssenkusu J, Kiguli S, Kalyesubula R, Lubega J, Mutebi EI, Opoka RO, John CC, Starr MC, Conroy AL
BMC Nephrol 2022 Mar 18;23(1):110. doi: 10.1186/s12882-022-02731-9. PMID: 35303803Free 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
Vitamin D supplementation for sickle cell disease.
Soe HH, Abas AB, Than NN, Ni H, Singh J, Said AR, Osunkwo I
Cochrane Database Syst Rev 2017 Jan 20;1(1):CD010858. doi: 10.1002/14651858.CD010858.pub2. PMID: 28105733Free PMC Article
Intravenous Lidocaine as an Adjuvant for Pain Associated with Sickle Cell Disease.
Nguyen NL, Kome AM, Lowe DK, Coyne P, Hawks KG
J Pain Palliat Care Pharmacother 2015;29(4):359-64. doi: 10.3109/15360288.2015.1082009. PMID: 26654408

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
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
Hydroxyurea and blood transfusion therapy for Sickle cell disease in South Asia: inconsistent treatment of a neglected disease.
Darshana T, Rees D, Premawardhena A
Orphanet J Rare Dis 2021 Mar 23;16(1):148. doi: 10.1186/s13023-021-01781-w. PMID: 33757549Free PMC Article
Preoperative blood transfusions for sickle cell disease.
Estcourt LJ, Fortin PM, Trivella M, Hopewell S
Cochrane Database Syst Rev 2016 Apr 6;4(4):CD003149. doi: 10.1002/14651858.CD003149.pub3. PMID: 27049331Free PMC Article

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