Sickle cell anemia is an inherited form of anemia caused by the inheritance of primarily

Hemoglobin S which is the most common type of abnormal hemoglobin and the basis of the

sickle cell trait and sickle cell anemia. The cause of this is due to the difference in structure of

Hemoglobin S from Hemoglobin A(regular hemoglobin) by which one of the structural

components, glutamine is replaced by valine in the 6th position of the beta chain of globin. Due

to this, normal red blood cells which possess a flexible and round shape which allows for easy

transportation through blood vessels instead possess a rigid and sickle shaped cell which due to

its shape may adhere to the walls of small blood vessels which may lead to the slowing down of

blood flow and oxygen deprivation to parts of the body. This essay aims to tackle the clinical

presentations and laboratory findings of a patient with suspected sickle cell anemia.

Within a patient who is suspected of possessing sickle cell anemia, many clinical manifestations

may appear. The most common of these clinical manifestations is vaso-occlusive crisis. A vaso-

occlusive crisis is a common painful complication of sickle cell anemia in adolescents and

adults. It is a form of sickle cell crisis. A sickle cell crisis is a painful episode that may begin

suddenly in a person who has sickle cell disease. A sickle cell crisis occurs when sickle-shaped

red blood cells clump together and block small blood vessels that carry blood to certain organs,

muscles, and bones. This causes mild to severe pain. A vaso-occlusive crisis occurs when the

microcirculation is obstructed by sickled RBCs, causing ischemic injury to the organ supplied

and resultant pain. Pain crises constitute the most distinguishing clinical feature of sickle cell

disease and are the leading cause of emergency department visits and hospitalizations for

affected patients. Some other major features and symptoms of sickle cell anemia which are

generally present within the patient are: fatigue, anemia, dactylitis (swelling of the hands and

feet), aseptic necrosis and bone infracts are amongst many.

Once clinical presentations have been seen, confirmatory tests are performed within the lab to

confirm the diagnosis of sickle cell anemia. There are many tests which are conducted to confirm

the presence of sickle cell anemia. The sickle cell test is a standard bloodtest by which is blood is

drawn and examined. The technician who examines the blood will be looking for the abnormal

form of hemoglobin, hemoglobin S. A sickle cell test looks only for the presence of hemoglobin S,

which causes SCD (sickle cell disease). A negative test is normal. It means that the patient’s hemoglobin

is normal. A positive test result may mean the patient has the sickle cell trait or SCD.

If the test is positive, the doctor will probably order a second test called hemoglobin

electrophoresis. This will help determine which condition the patient has. If the test shows that

the patient has two abnormal hemoglobin genes, they will most likely be diagnosed with sickle

cell disease. If the test shows that they have only one of these abnormal genes and no symptoms,

they will likely be diagnosed with having sickle cell trait. Other tests include the CBC (complete

blood count), which will show hemoglobin levels in the range of 6-8 g/dL with a high

reticulocyte count (greater than 1.5%) if sickle cell anemia is present. On a peripheral blood film,

one can observe features of hyposplenism i.e., target cells and Howell-Jolly bodies. Sickling of

the red blood cells, on a blood film, can be induced by the addition of sodium metabisulphite.

Another test is Sickle Solubility Test. A mixture of hemoglobin S (Hb S) in a reducing solution

e.g., sodium dithionite gives a turbid appearance while normal Hb gives a clear solution. Iron

studies may also be conducted on the patient. These may include: iron, ferritin, UIBC, TIBC, and

transferrin saturation. These tests measure different aspects of the body's iron storage and usage.

They are ordered to help determine whether someone has an iron deficiency anemia or an excess

amount of iron (iron overload). People with sickle cell anemia who receive multiple blood

transfusions may experience an iron overload.

In conclusion, the patient will demonstrate extreme clinical signs ranging from extreme pain

amongst many along as positive testing in various laboratory testing, all of which may lead to the

conclusion that sickle cell anemia is present.