Which of the following would be increased in sickle cell disease?

RUHS, Jaipur, PB B.Sc Nursing Entrance-2024

Pathology & Genetics

Hard

Appeared in: RUHS, Jaipur, PB B.Sc Nursing Entrance-2024

Explanation

Sickle cell disease is a form of chronic hemolytic anemia, where red blood cells are destroyed prematurely.
The bone marrow attempts to compensate for the chronic loss of red blood cells by increasing erythropoiesis (RBC production).
Reticulocytes are immature red blood cells, and their count increases significantly as the bone marrow releases them into circulation at a higher rate.
A sustained high reticulocyte count is a classic indicator of the bone marrow's response to ongoing hemolysis.

Option A: While the platelet count can be elevated (thrombocytosis) in sickle cell disease due to factors like chronic inflammation and functional asplenia, it is not the most direct or consistent indicator of the bone marrow's compensation for hemolysis.
Option B: Hematocrit is the percentage of blood volume occupied by red blood cells. Due to the chronic destruction of RBCs (hemolysis) in sickle cell disease, the hematocrit level is typically decreased, reflecting a state of anemia.
Option D: Hemoglobin is the protein in red blood cells that carries oxygen. Because of the constant hemolysis and resulting anemia, the overall hemoglobin level in individuals with sickle cell disease is characteristically low.

Clinical Relevance

Nursing practice connection: Knowing Laboratory findings in sickle cell disease helps nurses interpret findings accurately and avoid errors in routine assessment, medication administration, and patient teaching.
The reticulocyte count is a crucial lab value for nurses to monitor in patients with sickle cell disease. It provides insight into the bone marrow's ability to respond to anemia.
A sudden drop in the reticulocyte count in a patient with sickle cell disease is a red flag, as it may signal an aplastic crisis (often caused by parvovirus B19 infection), where the bone marrow temporarily stops producing red blood cells. This is a medical emergency requiring immediate intervention.
Patient education should include the signs of worsening anemia (e.g., extreme fatigue, pallor, shortness of breath) and the importance of seeking care, as this could indicate an aplastic or sequestration crisis.

How to Approach the Question

First, identify the core of the question: it asks for a laboratory value that is increased in sickle cell disease.
Recall the fundamental pathophysiology of sickle cell disease: it is a chronic hemolytic anemia. This means red blood cells are being destroyed at a high rate.
Consider the body's natural response to anemia. The bone marrow, the factory for blood cells, will ramp up production to try and compensate for the loss.
Evaluate each option in this context:
Hemoglobin/Hematocrit: These measure the amount of RBCs and their protein. Since RBCs are being destroyed, these values will be low.
Platelet count: This can be high due to inflammation, but it's not a direct response to RBC loss.

Concept Tested & Keywords

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E6 Ghai Essential Pediatrics(pp 26-904 of 913) p. 365-367