What is the most likely cause of worsening anemia, characterized by a decrease in hemoglobin from 8.6 g/dL to 6.5 g/dL, in an 18-year-old woman with Sickle Cell Disease (SCD) on hydroxyurea (Hydroxycarbamide) and folic acid?

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Differential Diagnosis for Worsening Fatigue in a Patient with Hemoglobin SS Disease

  • Single most likely diagnosis:
    • C. Medication adverse effect: The patient has been taking hydroxyurea, which is known to cause bone marrow suppression, leading to a decrease in hemoglobin levels and an increase in mean corpuscular volume (MCV). The decrease in reticulocytes also supports this diagnosis, as hydroxyurea can suppress erythropoiesis.
  • Other Likely diagnoses:
    • B. Hyperhemolytic crisis: Although the patient has not had any vasoocclusive pain crises, a hyperhemolytic crisis can occur without pain and can lead to a decrease in hemoglobin levels. However, the lack of an increase in reticulocytes and the presence of a decreased reticulocyte count make this diagnosis less likely.
    • A. Anemia of inflammation: Chronic inflammation can lead to anemia of inflammation, which can cause a decrease in hemoglobin levels. However, the patient's temperature is normal, and there is no other evidence of inflammation, making this diagnosis less likely.
  • Do Not Miss (ddxs that may not be likely, but would be deadly if missed.):
    • E. Viral-induced pure red cell aplasia: This is a rare but potentially life-threatening condition that can cause a severe decrease in red blood cell production. Although it is unlikely, it is essential to consider this diagnosis due to its potential severity.
    • F. Vitamin B12 deficiency: A vitamin B12 deficiency can cause a decrease in hemoglobin levels and an increase in MCV. Although the patient is taking folic acid, a vitamin B12 deficiency can still occur, and it is essential to rule out this diagnosis.
  • Rare diagnoses:
    • D. Myelodysplastic syndrome: This is a rare condition that can cause bone marrow failure, leading to a decrease in hemoglobin levels. However, it is unlikely in a patient with hemoglobin SS disease, and there is no other evidence to support this diagnosis.

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This information is intended for healthcare professionals. Any medical decision-making should rely on clinical judgment and independently verified information. The content provided herein does not replace professional discretion and should be considered supplementary to established clinical guidelines. Healthcare providers should verify all information against primary literature and current practice standards before application in patient care. Dr.Oracle assumes no liability for clinical decisions based on this content.

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