Vaso-occlusive crisis

A vaso-occlusive crisis is a common painful complication of sickle cell anemia in adolescents and adults.^[1][2] It is a form of sickle cell crisis. Sickle cell anemia – most common in those of African, Hispanic, and Mediterranean origin – leads to sickle cell crisis when the circulation of blood vessels is obstructed by sickled red blood cells, causing ischemic injuries. The most common complaint is of pain, and recurrent episodes may cause irreversible organ damage.^[2] One of the most severe forms is the acute chest syndrome which occurs as a result of infarction of the lung parenchyma. This can rapidly result in death. Other types of vaso-occlusive crisis in sickle cell anemia include dactylitis, priapism, abdominal pain, and jaundice.

Vaso-occlusive crisis
Specialty	Angiology
Symptoms	acute, severe pain
Diagnostic method	There is no test to confirm a vaso-occlusive crisis, but tests can be done to rule out other causes. Patients with vaso-occlusive crisis present with pain (mild to severe) and a history of sickle cell anemia.
Differential diagnosis	Neuropathic pain, hyperalgesia, osteomyelitis

Diagnosis

Diagnosis of vaso-occlusive crisis is based on clinical manifestations, complete blood count with white blood cell differential, platelet count, reticulocyte count, and comprehensive metabolic panel with liver and kidney function tests.^[3] Typical lab findings include acute drop in hemoglobin concentration, increased platelet count, increased reticulocyte count, and elevated serum urea.^[4]

Management

The management of an acute event of vaso-occlusive crisis is the use of potent analgesics (opioids),^[5] rehydration with normal saline or Ringer's lactate, treatment of malaria (whether symptomatic or not) using artemisinin combination therapy, and the use of oxygen via face mask, especially for acute chest syndrome. Hyperbaric oxygen has also been shown to be a useful adjunct in pain reduction.^[6]

References

1. Stuart MJ, Nagel RL (2004). "Sickle-cell disease". Lancet. 364 (9442): 1343–60. doi:10.1016/S0140-6736(04)17192-4. PMID 15474138. S2CID 8139305.
2. Lanzkron S, Carroll CP, Haywood C (October 2010). "The burden of emergency department use for sickle-cell disease: an analysis of the national emergency department sample database". Am. J. Hematol. 85 (10): 797–9. doi:10.1002/ajh.21807. PMC 3431910. PMID 20730795.
3. YAWN, BARBARA P.; JOHN-SOWAH, JOYLENE (15 December 2015). "Management of Sickle Cell Disease: Recommendations from the 2014 Expert Panel Report". American Family Physician. 92 (12) (12 ed.): 1069–1076A. PMID 26760593.
4. Rees, David C.; Gibson, John S. (28 November 2011). "Biomarkers in sickle cell disease". British Journal of Haematology. 156 (4): 433–445. doi:10.1111/j.1365-2141.2011.08961.x. PMID 22122125.
5. Campos J, Lobo C, Queiroz AM, do Nascimento EM, Lima CB, Cardoso G, Ballas SK (July 2014). "Treatment of the acute sickle cell vaso-occlusive crisis in the Emergency Department: a Brazilian method of switching from intravenous to oral morphine". Eur. J. Haematol. 93 (1): 34–40. doi:10.1111/ejh.12293. PMID 24571671. S2CID 28732478.
6. Stirnemann J, Letellier E, Aras N, Borne M, Brinquin L, Fain O (June 2012). "Hyperbaric oxygen therapy for vaso-occlusive crises in nine patients with sickle-cell disease". Diving Hyperb Med. 42 (2): 82–4. PMID 22828815. Archived from the original on July 9, 2014. Retrieved 2014-07-06.{{cite journal}}: CS1 maint: unfit URL (link)

External links