Sickle-cell disease history and symptoms
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]
Overview
Patients with sickle-cell anemia can have symptoms that vary in severity.
Symptoms
A vaso-occlusive crisis is caused by sickle-shaped red blood cells that obstruct capillaries and restrict blood flow to an organ, resulting in ischemia, pain, and organ damage.
Because of its narrow vessels and function in clearing defective red blood cells, the spleen is frequently affected. It is usually infarcted before the end of childhood in individuals suffering from sickle-cell anemia. This autosplenectomy increases the risk of infection from encapsulated organisms;[1][2] preventive antibiotics and vaccinations are recommended for those with such asplenia.
Bones, especially weight-bearing bones, are also a common target of vaso-occlusive damage. This is due to bone ischemia.
A recognised type of sickle crisis is the acute chest crisis, a condition characterised by fever, chest pain, hard breathing, and pulmonary infiltrate on chest X-ray. Given that pneumonia and intrapulmonary sickling can both produce these symptoms, the patient is treated for both conditions.
Other sickle-cell crises
- Aplastic crises are acute worsenings of the patient's baseline anemia producing pallor, tachycardia, and fatigue. This crisis is triggered by parvovirus B19, which directly affects erythropoiesis (production of red blood cells). Parvovirus infection nearly completely prevents red blood cell production for 2-3 days. In normal individuals this is of little consequence but the shortened red cell life of sickle-cell patients results in an abrupt, life-threatening situation. Reticulocyte counts drop dramatically during the illness and the rapid turnover of red cells leads to the drop in hemoglobin. Most patients can be managed supportively; some need blood transfusion.
- Splenic sequestration crises are acute, painful enlargements of the spleen. The abdomen becomes bloated and very hard. Management is supportive, sometimes with blood transfusion.
References
- ↑ Pearson H. "Sickle cell anemia and severe infections due to encapsulated bacteria". J Infect Dis. 136 Suppl: S25–30. PMID 330779.
- ↑ Wong W, Powars D, Chan L, Hiti A, Johnson C, Overturf G (1992). "Polysaccharide encapsulated bacterial infection in sickle cell anemia: a thirty year epidemiologic experience". Am J Hematol. 39 (3): 176–82. PMID 1546714.