Question

Anemias Case Study

A 5 year old son of a Liberian exchange student was hospitalized because of severe diarrhea, abdominal distention and splenomegaly. On admission the following tests were ordered: CBC, bilirubin, hemoglobin electrophoresis, electrolyte studies, urinalysis and stool culture. The results of these tests were as follows:

WBC: 8,700/ul

RBC: 2.06 x 10⁶/ul

Hb: 5.8 g/dl

HCT: 19.6%

MCV: 96.4 fl

MCHC: 32 g/dl

PLT: 420,000/ul

The peripheral blood smear revealed moderate aniso, 4 + poik, 3+ poly, occasional HJ bodies, 2+ drepanocytes, and 2 + codocytes and 12 NRBCs. The patient’s total bilirubin was 6.0 mg/dl. The patient had severely abnormal electrolyte levels.

Questions:

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

2. What is the clinical course of this disease?

3. Explain the presence of the drepanocytes and NRBCs on the peripheral blood smear.

4. What additional tests should be ordered to confirm your diagnosis?

5. What kind of treatment should this patient receive?

Answer 1

Causes of patient's condition:

• On the above laboratory test the client is diagnosed with "SICKLE CELL ANEMIA". The patient complain severe diarrhea,abdominal distension and splenomegaly on admission. Episodes of pain called as pain crisis it is major symptom the sickle cell anemia.
• All the above mentioned complaints are symptoms for sickle cell anemia so exact trigger mechanism was not known,dehydration,emotional stress,fatigue and decrease level of hemoglobin can cause the pain crisis episodes in patient with anemia.

Clinical course of sickle cell anemia

• It takes a chronic clinical course.The course of sickle cell anemia that can characterized by hemolytic episodes,severe organ damage such as liver, kidney that can be caused by decrease blood flow to the organs that deprive the blood and oxygen to the affected organs.
• In sickle cell anemia the oxygen and nutrient level become low that can damage organs and can be fatal.Pain crisis is a feature for sickle cell anemia.The crisis that can obstruct the blood flow to the micro circulation of brain that may leads to stroke an to the eyes cause blindness and sometimes consequent necrosis of tissue.

Presence of drepanocytes and Nucleated RBCs on the peripheral blood smear

• In blood smear derpanocytes are elongated and crescent shaped red blood cells that can be characteristic feature of anemia as well as hemoglobin S-thalsemmia.Obstruction in the micro circulation cause decrease oxygen level in tissues and lowering of blood pH that promote the shape of hemoglobin-S.By the use of gelation,the sickle cell anemia assume a discoid shape following oxygenation.Sickle cell anemia cell will not regain their normal shape
• Nucleated RBCs are immature blood cell that aren't present in blood that are produced from the bone marrow after that can released in to circulation.These cell may be seen in the severe hemolysis.Polychromai absence is indication for aplastic crisis.

Additional test for sickle cell anemia

• Ultrasound is a painless test that is used to measure the blood flow and to prevent the heart disease.
• Amniotic fluid used to diagnosis sickle cell anemia before birth in unborn baby.
• Shape of cell by blood smear.

5.Treatment for patient with sickle cell anemia

Treatment include blood transfusion and medication

Medication: If available in hospital

• Hydroxyurea(15mg/kg/day) is used to reduces the frequency of pain crisis and need blood transfusion.Contraindication for pregnancy.Side effects:Neutropenia,Ulcer and gangrene
• L- glutamine oral powder(5g,10g,15g orally twice daily) used for sickle cell anemia and help in reducing frequency of pain.Side effects:Constipation, Headache,depression.
• Crizanlizumab (5mg/kg IV infusion over 30minutes) is used for sickle anemia. Side effects nausea, Fever, backache.
• Voxelotor(1500mg orally once daily) used for anemia.Side effects:Fatigue,nausea,vomiting
• Oral rehydration therapy for diarrhea.
• Vitamin supplements to increase hemoglobin level.

Blood transfusion which help to reduce symptoms and complications

Stem cell transplant