Sickle-cell disease history and symptoms
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1] Shyam Patel [2]
Overview
Patients with sickle cell anemia can have symptoms that vary in severity. Symptoms vary from mild pain in the arms an legs to severe pain from sickle cell crises and acute chest syndrome.
Symptoms
The reason for most symptoms of sickle cell disease is vaso-occlusive crises and deoxygenated hemoglobin. 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.
The location of vaso-occlusive episodes determines the types of symptoms a patient will experience. For example, vaso-occlusive crisis in the bones will cause bone pain. Bones, especially weight-bearing bones, are a common target of vaso-occlusive damage due to bone ischemia. Dactylitis can occur in children since the small bones of the extremities have bone marrow. Vaso-occlusive in the eyes will cause blurry vision or eye pain due to retinal artery of vein occlusion. Vaso-occlusive episodes in the penis will cause priapism, a persistent and painful erection due to obstruction of penile vasculature. Vaso-occlusive in the brain can cause stroke, which can present with a variety of symptoms such as numbness, tingling, motor loss, speech deficits, or gait disturbance. Vaso-occlusive in the limbs can cause leg ulceration.
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.
A recognized 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.
Symptoms that result from deoxygenated hemoglobin and anemia include fatigue, shortness of breath, and pallor.
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 blood 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.