U.S. flag

An official website of the United States government

Format

Send to:

Choose Destination

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

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
Evidence-based management of sickle cell disease in the emergency department.
Glassberg J
Emerg Med Pract 2011 Aug;13(8):1-20; quiz 20. PMID: 22164362
Management of painful vaso-occlusive crisis of sickle-cell anemia: consensus opinion.
Mousa SA, Al Momen A, Al Sayegh F, Al Jaouni S, Nasrullah Z, Al Saeed H, Alabdullatif A, Al Sayegh M, Al Zahrani H, Hegazi M, Al Mohamadi A, Alsulaiman A, Omer A, Al Kindi S, Tarawa A, Al Othman F, Qari M
Clin Appl Thromb Hemost 2010 Aug;16(4):365-76. Epub 2010 Jun 7 doi: 10.1177/1076029609352661. PMID: 20530056

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

Economic and Clinical Burden of Managing Sickle Cell Disease with Recurrent Vaso-Occlusive Crises in the United States.
Udeze C, Evans KA, Yang Y, Lillehaugen T, Manjelievskaia J, Mujumdar U, Li N, Andemariam B
Adv Ther 2023 Aug;40(8):3543-3558. Epub 2023 Jun 18 doi: 10.1007/s12325-023-02545-7. PMID: 37332020Free 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
Management of painful vaso-occlusive crisis of sickle-cell anemia: consensus opinion.
Mousa SA, Al Momen A, Al Sayegh F, Al Jaouni S, Nasrullah Z, Al Saeed H, Alabdullatif A, Al Sayegh M, Al Zahrani H, Hegazi M, Al Mohamadi A, Alsulaiman A, Omer A, Al Kindi S, Tarawa A, Al Othman F, Qari M
Clin Appl Thromb Hemost 2010 Aug;16(4):365-76. Epub 2010 Jun 7 doi: 10.1177/1076029609352661. PMID: 20530056

Therapy

Apadamtase Alfa: First Approval.
Heo YA
Drugs 2024 Apr;84(4):467-472. doi: 10.1007/s40265-024-02007-6. PMID: 38418772
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
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

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

Supplemental Content

Table of contents

    Practice guidelines

    • PubMed
      See practice and clinical guidelines in PubMed. The search results may include broader topics and may not capture all published guidelines. See the FAQ for details.
    • Bookshelf
      See practice and clinical guidelines in NCBI Bookshelf. The search results may include broader topics and may not capture all published guidelines. See the FAQ for details.

    Related information

    • PMC Articles
      Related information in PubMed Central Links
    • PubMed
      Related literature resources in PubMed

    Recent activity

    Clear Turn Off Turn On

    Your browsing activity is empty.

    Activity recording is turned off.

    Turn recording back on

    See more...
    -