Case Study

A 55-year-old woman complained to her physician that her finger and toes became blue during cold weather. When she warmed them up, her digits became painful. She also noted that she has been feeling extremely fatigued, with tachycardia and dyspnea. There was no family history of anemia or any other inherited hemato-logical condition, but there has been a history of vascular disease in her paternal side. A CBC and differential were ordered with the following results: WBC 8.0 X 10 9/L, RBC 3.04 X 1012/L, Hgb 9.0 g/dL, Hct 28.0%, MCV 82 fL, MCH 28 pg, and MCHC 30.2%. A reticulocyte count was ordered once the CBC was performed. Which anemic condition can lead to this patient's unusual symptoms?

Insights to the Case Study

This patient is showing signs of anemia with a low RBC, Hgb, and Hct. Additionally, she is showing the physical symptoms of anemia, which include shortness of breath (dyspnea), heart palpitations (tachycardia), and fatigue.

Her symptoms related to her fingers and toes suggest Raynaud's syndrome, and her physician proceeded to order a direct antiglobulin test battery using all three antihuman globulin reagents. This test was positive with agglutination in the complement anti-human globulin reagent, indicating complement coating of the red cells. Her physician diagnosed cold agglutinin syndrome in the early stages. Cold agglutinin syndrome is a hemolytic anemia most often associated with cold reactive autoanti-bodies, which are complement binding. This syndrome accounts for approximately 16% to 23% of all cases of immune hemolytic processes. Patients experience hemol-ysis and hemoglobinuria at cold temperatures as well as the physical symptoms indicated in this case. Many individuals move to warmer climates to prevent hemolytic episodes or if symptoms exacerbate. In addition, our patient's peripheral blood smear showed occasional sphe-rocytes and moderate polychromasia, and her reticulo-cyte count was 3.0% (normal value, 0.5% to 1.5%).

48 Part I • Basic Hematology Principles

Review Questions _

1. What is a significant morphological difference between irreversibly sickled cells and reversible sickled cells?

a. Puddled hemoglobin b. Crystal formation central to the sickle cells c. Pointed projections to the sickle cell d. Fragmentation of the red cell membrane

2. What are two integral proteins in the red cell structure that house red cell antigens?

a. Glycoproteins and glycolipids b. Glycophorin A and glycophorin B

c. Cholesterol and spectrin d. Sodium and potassium

3. All of the following are characteristic of the red cell in stages of development except a. nuclei are "baseball" round.

b. immature cells are larger.

c. N:C ratio decreases as the cell matures.

d. distinct granulation in the cytoplasm.

4. Which red cell inclusions originate as a result of denatured hemoglobin?

a. Howell-Jolly bodies b. Heinz bodies c. Pappenheimer bodies d. Malarial parasites

5. In which conditions can you see elliptocytes?

a. Iron loading processes b. Sickle cell anemia c. Iron deficiency anemia d. Thalassemia

6. Which red cell morphology may form as a result of excess cholesterol taken upon the red cell membrane?

a. Macrocytes b. Target cells c. Schistocytes d. Ovalocytes

7. Hypochromia is used to define a. color change in the red cell.

b. variation in shape of the red cell.

c. variation in size of the red cell.

d. decrease in hemoglobin content of the red cell.

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