Thalassemia: Diagnosis (Pediatric Nursing)

by Paula Ruedebusch

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    00:01 Moderate and severe thalassemias are often diagnosed in childhood because these symptoms usually appear in the first two years of life.

    00:07 So what do we do to diagnose a patient? Well you do a CBC.

    00:11 This includes a measure of hemoglobin and the quantity and size of the red blood cells Patients with thalassemia have fewer healthy red blood cells and less hemoglobin than normal patients and those with alpha and beta thalassemia trait may have smaller than normal red blood cells and these are microcytic.

    00:28 A reticulocyte count is a measure of the young red blood cells in circulation and this can indicate that your bone marrow is not producing an adequate number of red blood cells.

    00:37 The RDW may assist in differentiating iron deficiency and sideroblastic anemia from thalassemia.

    00:44 The RDW is going to be elevated in more than 90% of patients with iron deficiency anemia but this is only seen in about 50% of patients with thalassemia.

    00:54 An iron panel is next and this is key, this is where you find your difference.

    00:59 An iron panel's gonna be performed because this will differentiate iron deficiency anemia from thalassemia.

    01:05 These are different conditions and on a CBC, they can look the same.

    01:09 The management of the two conditions is very different.

    01:13 Next you can do hemoglobin electrophoresis and this can be used to help diagnose beta thalassemia.

    01:18 And finally there are genetic test available.

    01:21 Here we see a CBC, this includes components of a red blood cell count, the white blood cell count and you'll also see a differential, this is the percentage of each type of white blood cell.

    01:30 You'll get a platelet count, the reticulocyte distribution width which again is the size of the red blood cells, hematocrit and this is the red blood cell percentage for blood volume, and also the hemoglobin.

    01:43 These are the red blood cell indices that can be used to describe the red blood cells size and color.

    01:48 the MCV is the cell size.

    01:51 We also get the MCHC and this is a ratio.

    01:55 And next we have the mean cell hemoglobin and this is the amount of hemoglobin on the cell.

    02:00 Here we see our indices and at the bottom you'll see the MCV.

    02:04 Remember, this is the red blood cell size and you can describe this in three different ways.

    02:08 The cell can be microcytic where it's too small, normocytic is a normal size and macrocytic, you have this abnormally large red blood cells.

    02:17 Next on the top left, you'll see the MCH, remember this is the amount of hemoglobin in the cell, this provides the color.

    02:23 If it's hypochromic, means low color, there is not enough hemoglobin.

    02:27 If it's normochromic, there's a normal amount of hemoglobin.

    02:30 And there's no such thing as hyperchromic, we don't worry about a cell having too much hemoglobin.

    02:36 Next we're gonna do an iron panel and remember this is key in differentiating iron deficiency anemia from thalassemia.

    02:43 First we'll do the iron level, this is just the amount of iron carried by transferrin.

    02:48 We also have the transferrin level and this is a transport protein used to move iron through the body.

    02:54 It's produced by the liver and in patients with liver dysfunction, they're going to have a decreased transferrin production.

    03:00 Next we have ferritin and this is the amount of stored iron in the body.

    03:04 Patients will have an artificially high ferritin in the settings of infection, late stage cancer, severe inflammation and alcoholism.

    03:13 Now we have the TIBC or total iron binding capacity, and this is a tricky one to conceptualize.

    03:19 This is a measure of the open spots for iron to bind, it's not a measure of the amount of iron that is bound.

    03:26 So if a patient has a low iron level, they're going to have a lot of available binding sites available from where iron's attached.

    03:33 Here we can compare the differences between the lab findings of a patient with thalassemia and a patient with iron deficiency anemia.

    03:40 remember on a CBC, the two conditions can initially mimic each other so the clinician needs to delve deeper with an iron panel.

    03:47 In both conditions, the anemia can range from minor to severe, In both conditions, a microcytic anemia will be noted with the cells having a slightly lower MCV or volume in thalassemia when compared to iron deficiency anemia The MCH, both conditions will reveal a hypochromic cell or a pale cell Here is the major difference - the serum iron.

    04:11 In thalassemia, it is increased.

    04:13 So you want to be sure that you differentiate this condition from iron deficiency anemia because the treatment of iron deficiency anemia is to give your patient iron.

    04:21 But in a patient with thalassemia, you do not want to add in any extra iron because that's gonna cause lots of problems.

    04:28 In iron deficiency anemia, the serum level is low requiring supplementation.

    04:34 TIBC, this is also normal in patients with thalassemia but it's going to be increased in your patients with iron deficiency anemia.

    04:42 Remember this refers to the open spots available for iron to bind and a person who is deficient in iron has a lot of room for more to bind so this level's going to be increased in patients with iron deficiency anemia.

    04:54 The patient with thalassemia already has enough iron, they don't need anymore open binding spots.

    05:00 The marrow iron stores, now this has to do a lot with how much iron is stored in the marrow.

    05:04 This level is increased in patients with thalassemia and of course these stores are absent in patients who are iron deficient because they don't have any extra iron to stash away.

    05:15 The serum ferritin, it's gonna be much higher in a patient with thalassemia.

    05:19 This is the iron stored in the blood and this is an easy distinguisher between the two conditions when comparing the lab values.

    05:26 The bilirubin, this level's going to be increased in patients with thalassemia as there is increased hemolysis or the breakdown of the red blood cells.

    05:34 Bilirubin is a byproduct of this and there's gonna be a normal level of bilirubin in patients with iron deficiency anemia because this process is not as destructive.

    05:44 The RDW, this is short for red blood cell distribution width and this is a distribution of the sizes of the red blood cells.

    05:51 This is gonna be normal in the setting of thalassemia.

    05:54 In patient with iron dificiency anemia, their red blood cell sizes are going to vary greatly so an elevated RDW is the hallmark of iron deficiency anemia.

    About the Lecture

    The lecture Thalassemia: Diagnosis (Pediatric Nursing) by Paula Ruedebusch is from the course Blood Disorders – Pediatric Nursing. It contains the following chapters:

    • Diagnosis
    • Thalassemia vs. Iron Deficient Anemia

    Included Quiz Questions

    1. Mean cell volume (MCV)
    2. Hemoglobin (Hgb)
    3. Hematocrit (Hct)
    4. Mean cell hemoglobin (MCH)
    1. Increased
    2. Slightly decreased
    3. Normal
    4. Very low
    1. Normal
    2. Increased
    3. Very low
    4. Decreased

    Author of lecture Thalassemia: Diagnosis (Pediatric Nursing)

     Paula Ruedebusch

    Paula Ruedebusch

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