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==Overview==
==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 of sickle-cell disease include 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 nociception|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.
==History and Symptoms==
The most common symptoms of sickl cell disease are bone pain, [[numbness]] and [[tingling]].


Because of its narrow vessels and function in clearing defective red blood cells, the [[spleen]] is frequently affected. It is usually [[infarction|infarcted]] before the end of childhood in individuals suffering from sickle-cell anemia. This  [[autosplenectomy]] increases the risk of infection from [[encapsulated organisms]];<ref>{{cite journal |author=Pearson H |title=Sickle cell anemia and severe infections due to encapsulated bacteria |journal=J Infect Dis |volume=136 Suppl |issue= |pages=S25-30 |year= |pmid=330779}}</ref><ref>{{cite journal |author=Wong W, Powars D, Chan L, Hiti A, Johnson C, Overturf G |title=Polysaccharide encapsulated bacterial infection in sickle cell anemia: a thirty year epidemiologic experience |journal=Am J Hematol |volume=39 |issue=3 |pages=176-82 |year=1992 |pmid=1546714}}</ref> preventive antibiotics and vaccinations are recommended for those with such [[asplenia]].
==History==
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.


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==
Common symptoms of sickle cell disease include:<ref name="pmid11452073">{{cite journal |vauthors=Lonergan GJ, Cline DB, Abbondanzo SL |title=Sickle cell anemia |journal=Radiographics |volume=21 |issue=4 |pages=971–94 |date=2001 |pmid=11452073 |doi=10.1148/radiographics.21.4.g01jl23971 |url=}}</ref><ref name="pmid12112099">{{cite journal |vauthors=Prengler M, Pavlakis SG, Prohovnik I, Adams RJ |title=Sickle cell disease: the neurological complications |journal=Ann. Neurol. |volume=51 |issue=5 |pages=543–52 |date=May 2002 |pmid=12112099 |doi=10.1002/ana.10192 |url=}}</ref><ref name="pmid24167104">{{cite journal |vauthors=Brandow AM, Farley RA, Panepinto JA |title=Neuropathic pain in patients with sickle cell disease |journal=Pediatr Blood Cancer |volume=61 |issue=3 |pages=512–7 |date=March 2014 |pmid=24167104 |pmc=4357477 |doi=10.1002/pbc.24838 |url=}}</ref>
*[[Pain and nociception|Pain]] and/or [[bone pain]]
*[[Dactylitis]]
*[[Blurry vision]]
*[[priapism|Persistent and painful erection]]
*[[Numbness]]
*[[Tingling]]
*Motor skill loss
*[[aphasia|Speech deficits]]
*[[Gait disturbance]]
*Leg ulceration
*[[Jaundice]]


Symptoms that result from deoxygenated hemoglobin and anemia include fatigue, shortness of breath, and pallor.
===Acute chest crisis===
 
Symptoms of acute chest crisis include:<ref name="pmid23534969">{{cite journal |vauthors=Desai PC, Ataga KI |title=The acute chest syndrome of sickle cell disease |journal=Expert Opin Pharmacother |volume=14 |issue=8 |pages=991–9 |date=June 2013 |pmid=23534969 |doi=10.1517/14656566.2013.783570 |url=}}</ref><ref name="pmid9057664">{{cite journal |vauthors=Vichinsky EP, Styles LA, Colangelo LH, Wright EC, Castro O, Nickerson B |title=Acute chest syndrome in sickle cell disease: clinical presentation and course. Cooperative Study of Sickle Cell Disease |journal=Blood |volume=89 |issue=5 |pages=1787–92 |date=March 1997 |pmid=9057664 |doi= |url=}}</ref>
===Other sickle-cell crises===
*[[Fever]]
*'''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.
*[[Chest pain]]
*'''Splenic sequestration crises''' are acute, painful enlargements of the spleen. The abdomen becomes bloated and very hard. Management is supportive, sometimes with blood transfusion.
*[[Labored breathing]]
*[[Fatigue]]  
*[[Shortness of breath]]
*[[Pallor]]


==References==
==References==
{{reflist|2}}
{{reflist|2}}
{{WH}}
{{WS}}
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Latest revision as of 15:12, 6 August 2018

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Shyam Patel [2]

Overview

Symptoms of sickle-cell disease include mild pain in the arms an legs to severe pain from sickle cell crises and acute chest syndrome.

History and Symptoms

The most common symptoms of sickl cell disease are bone pain, numbness and tingling.

History

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.

Symptoms

Common symptoms of sickle cell disease include:[1][2][3]

Acute chest crisis

Symptoms of acute chest crisis include:[4][5]

References

  1. ↑ Lonergan GJ, Cline DB, Abbondanzo SL (2001). "Sickle cell anemia". Radiographics. 21 (4): 971–94. doi:10.1148/radiographics.21.4.g01jl23971. PMID 11452073.
  2. ↑ Prengler M, Pavlakis SG, Prohovnik I, Adams RJ (May 2002). "Sickle cell disease: the neurological complications". Ann. Neurol. 51 (5): 543–52. doi:10.1002/ana.10192. PMID 12112099.
  3. ↑ Brandow AM, Farley RA, Panepinto JA (March 2014). "Neuropathic pain in patients with sickle cell disease". Pediatr Blood Cancer. 61 (3): 512–7. doi:10.1002/pbc.24838. PMC 4357477. PMID 24167104.
  4. ↑ Desai PC, Ataga KI (June 2013). "The acute chest syndrome of sickle cell disease". Expert Opin Pharmacother. 14 (8): 991–9. doi:10.1517/14656566.2013.783570. PMID 23534969.
  5. ↑ Vichinsky EP, Styles LA, Colangelo LH, Wright EC, Castro O, Nickerson B (March 1997). "Acute chest syndrome in sickle cell disease: clinical presentation and course. Cooperative Study of Sickle Cell Disease". Blood. 89 (5): 1787–92. PMID 9057664.
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