The 5-year-old son of a Liberian exchange student washospitalized because of severe diarrhea, abdominal distention, andsplenomegaly. The child had been previously diagnosed in Europe assuffering from sickle cell anemia and had experienced severalepisodes of sickle cell crisis. On admission, the following testswere ordered: CBC, platelet count, bilirubin determination,hemoglobin electrophoresis, Hb S screening test, electrolytestudies, urinalysis, and stool culture.

The results of the tests were as follows:

Hemoglobin 5.8 g/dL

Hct 19%

RBC 2.0 X10¹²/L

The RBC indices were as follows:

MCV 96 fL

MCH 29 pg

MCHC 31 g/dL

The total WBC count was 8.7X10⁹/L. The peripheralblood smear revealed moderate anisocytosis, macrocytosis,microcytosis, poikilocytosis, polychromatophilia, occasionalHowell-Jolly bodies, moderate basophilic stippling, many sickledRBCs (drepanocytes), and 12 (nucleated RBCs) metarubricytes/100WBCs. The distribution of platelets was normal on the blood smear,and the total platelet count was 0.42X 10¹²/L. Thepatient’s total serum bilirubin was 6.0 mg/dL. Hemoglobinelectrophoresis demonstrated Hb S 78% and Hb F 22%. The Hb Sscreening test result was positive. A routine urinalysis waspositive for occult blood, and the stool culture was normal. Thepatient had severely abnormal electrolytes.

1. What is your diagnosis of this case?

2. What is the cause of this patient’s condition?

3. What are the clinical symptoms commonly show patients of thisdisease?

4. Explain the presence of the drepanocytes on the peripheralblood film.