Clinical Manifestations

The clinical manifestations of sickle cell disease involve every organ system (Table 1). The most common presentations are acute pain crises, cerebral vascular accidents (CVA), acute chest syndrome (ACS), cholelithiasis, and splenic infarctions. Acute pain crisis is an episode of acute pain not caused by any other factor than sickle cell. It occurs in the vast

TABLE 1 Systemic Features of Sickle Cell Anemia

Organ system



Acute pain crisis

Cerebral vascular disease

Chronic pain syndrome


Acute chest syndrome

Reactive airway disease

Chronic restrictive lung disease




Chronic hepatitis (secondary to multiple blood transfusions)


Hemolytic anemia

Acute aplastic anemia

Splenic enlargement/autoinfarction




(Salmonella and Staphylococcus aureus)



Erythrocyte auto/alloimmunizations

Transfusion reactions

majority of these patients. The most common sites affected are the abdomen, femoral shaft, knee, and lower back. The etiology remains unclear. There appears to be an inciting factor in many cases. Inciting factors may include emotional stress, physical exertion, and alcohol consumption. Pain probably results from microinfarction of the end organ, for example, medullary infarction resulting in bone pain. About 1% of sickle cell patients have more than six episodes per year (12).

ACS is another common presentation of sickle cell crisis. Patients present with a new infiltrate on chest films, chest pain, fever, and tachypnea. The causes include infections (e.g., parvovirus), fat embolism from bone infarction, and surgery. This condition also causes chronic lung inflammation not unlike asthma in the early stages and restrictive lung diseases in the late stages (13).

CVA can be a devastating consequence of sickle cell. They may be thromboembolic or hemorrhagic. CVA associated with sickle cell disease may occur at any age, and often the first episode occurs in the first decade of life. Risk factors for CVA include being a homozygous genotype, history of transient ischemic attacks, and previous ACS episodes (14,15).

Splenic autoinfarction is another important clinical manifestation. This leads to increased susceptibility to infections from encapsulated organisms such as Haemophilus influenzae and Streptococcus pneumoniae (11).

Although these patients are at increased risk of infections due to pneumococcus and H. influenzae, there does not appear to be an increased risk of otitis media or sinusitis. Sickle cell patients do appear to be at increased risk for sleep-related breathing disorders. Sleep disturbance can be due to adenotonsillar hypertrophy or chronic lung disease.

Was this article helpful?

0 0
Beat The Battle With The Bottle

Beat The Battle With The Bottle

Alcoholism is something that can't be formed in easy terms. Alcoholism as a whole refers to the circumstance whereby there's an obsession in man to keep ingesting beverages with alcohol content which is injurious to health. The circumstance of alcoholism doesn't let the person addicted have any command over ingestion despite being cognizant of the damaging consequences ensuing from it.

Get My Free Ebook

Post a comment