NEET-PG 2017

Question 11: The patient with the following hematological abnormality presents with severe chest pain and difficulty in breathing. All are useful for managing this emergency except:

• A. Partial exchange transfusion
• B. Morphine
• C. Hydroxyurea
• D. Packed RBC (Correct Answer)

Explanation: ***Packed RBC*** - Transfusing **packed red blood cells** will increase the overall hemoglobin concentration, which can paradoxically increase blood viscosity and worsen vaso-occlusion in sickle cell crisis, especially in the setting of acute chest syndrome where fluid overload is a concern. - This is generally avoided as a first-line treatment for acute chest syndrome in sickle cell anemia unless the patient has severe anemia with a hemoglobin level significantly below their baseline. *Partial exchange transfusion* - **Partial exchange transfusion** is a critical intervention during sickle cell crises with severe complications like acute chest syndrome. - It rapidly reduces the percentage of **sickle hemoglobin (HbS)** and concurrently provides normal red blood cells, improving oxygen-carrying capacity and reducing blood viscosity without significantly increasing total red blood cell mass. *Morphine* - **Morphine** is essential for effective pain management in sickle cell crisis, including the severe chest pain associated with acute chest syndrome. - Adequate pain control helps reduce patient distress and can indirectly prevent worsening of the crisis by reducing stress-induced sympathetic activation. *Hydroxyurea* - **Hydroxyurea** is a long-term disease-modifying therapy for sickle cell anemia, increasing the production of **fetal hemoglobin (HbF)**, which reduces sickling. - While crucial for preventing future crises and complications, it is not an acute treatment for an emergency like acute chest syndrome, as its effects take weeks to months to manifest.