A 20 year old female presenting with anemia, mild jaundice for 2 years, peripheral smear showing spherocytes, the best investigation to be done is:
**Core Concept**
The patient's clinical presentation of anemia, mild jaundice, and spherocytes on peripheral smear is suggestive of a hemolytic anemia, specifically a hereditary condition. This condition is caused by the deficiency or dysfunction of the enzyme responsible for the breakdown of the red blood cell membrane.
**Why the Correct Answer is Right**
The patient's symptoms are consistent with Hereditary Spherocytosis (HS), a genetic disorder characterized by the production of abnormal red blood cells that are more prone to hemolysis. The correct investigation to confirm this diagnosis is the **Reticulocyte Count**, which measures the number of reticulocytes (immature red blood cells) in the peripheral blood. This helps to assess the bone marrow's ability to compensate for the increased red blood cell destruction. Additionally, a **Reticulocyte Count** can be used to monitor the effectiveness of treatment.
**Why Each Wrong Option is Incorrect**
**Option A:** A Coombs test (Direct Antiglobulin Test) is used to diagnose autoimmune hemolytic anemia, not hereditary spherocytosis.
**Option B:** A liver function test may reveal elevated bilirubin levels, but it does not directly diagnose the underlying cause of hemolysis.
**Option C:** A complete blood count (CBC) may show anemia, but it does not provide specific information about the underlying cause of the anemia.
**Clinical Pearl / High-Yield Fact**
Remember that Hereditary Spherocytosis is often associated with other genetic disorders, such as Beta-Thalassemia. A high index of suspicion is required for early diagnosis and treatment to prevent complications.
**Correct Answer:** C. Reticulocyte Count