Question:
High-performance liquid chromatography (HPLC) shows HbS very high with HbA around 40%. What is the most likely diagnosis?
High-performance liquid chromatography (HPLC) shows HbS very high with HbA around 40%. What is the most likely diagnosis?
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Always remember the golden rule for interpreting HPLC or electrophoresis:
If HbA $>$ HbS, it is Sickle Cell Trait (AS).
If HbS $>$ HbA, it is a disease state: either transfused Sickle Cell Anemia (SS) or Sickle Beta$^+$ Thalassemia (where beta chain production is suppressed).
If HbA is 0%, it is pure HbSS or Sickle Beta$^0$ Thalassemia.
If HbA $>$ HbS, it is Sickle Cell Trait (AS).
If HbS $>$ HbA, it is a disease state: either transfused Sickle Cell Anemia (SS) or Sickle Beta$^+$ Thalassemia (where beta chain production is suppressed).
If HbA is 0%, it is pure HbSS or Sickle Beta$^0$ Thalassemia.
Updated On: Sep 3, 2026
- Sickle cell disease (HbSS)
- Sickle cell trait (HbAS)
- Beta-thalassemia trait
- HbC disease
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The Correct Option is A
Solution and Explanation
Concept:
The question provides the results of a High-Performance Liquid Chromatography (HPLC) test, which is used for hemoglobin phenotyping.
The results show a "very high" level of Hemoglobin S (HbS) combined with Hemoglobin A (HbA) at approximately 40%.
We must correlate these findings with the correct hematologic diagnosis.
Explanation:
• To interpret hemoglobin electrophoresis or HPLC, one must understand the expected fractions in different conditions.
• Normal adult blood contains predominantly HbA ($>$ 95%), with minor amounts of HbA2 (1-3%) and HbF ($<$ 1%).
• In Sickle Cell Trait (HbAS), the patient is heterozygous. Because the normal beta-globin allele produces hemoglobin slightly more efficiently than the mutated allele, the amount of HbA is always greater than HbS.
• A typical HPLC for sickle cell trait shows about 60% HbA and 40% HbS.
• In the prompt, HbS is described as "very high" (implying it is the major fraction, $>$ 50%) while HbA is the minority at around 40%.
• Since HbS $>$ HbA, this completely rules out Sickle Cell Trait (Option B).
• In classic, un-transfused Sickle Cell Disease (HbSS, homozygous mutation), the patient produces absolutely no normal beta-globin. Therefore, their HPLC will show roughly 90-95% HbS, some HbF, normal or slightly elevated HbA2, and 0% HbA.
• How can a patient with HbSS have 40% HbA? This occurs when the patient has recently received a blood transfusion (usually an exchange transfusion for complications like acute chest syndrome or stroke prevention).
• The transfused normal donor blood introduces HbA into circulation.
• The target for chronic exchange transfusions in HbSS is typically to reduce the HbS fraction to below 30-50%, resulting in a significant presence of HbA (e.g., 40-60%).
• Given the options, this profile represents a transfused patient with Sickle cell disease (HbSS).
• Beta-thalassemia trait presents with elevated HbA2 ($>$ 3.5%) and microcytic anemia, not HbS.
• HbC disease involves Hemoglobin C, not Hemoglobin S.
Final answer:
The pattern of HbS being the dominant hemoglobin alongside 40% HbA strongly suggests transfused Sickle Cell Disease (HbSS).
The question provides the results of a High-Performance Liquid Chromatography (HPLC) test, which is used for hemoglobin phenotyping.
The results show a "very high" level of Hemoglobin S (HbS) combined with Hemoglobin A (HbA) at approximately 40%.
We must correlate these findings with the correct hematologic diagnosis.
Explanation:
• To interpret hemoglobin electrophoresis or HPLC, one must understand the expected fractions in different conditions.
• Normal adult blood contains predominantly HbA ($>$ 95%), with minor amounts of HbA2 (1-3%) and HbF ($<$ 1%).
• In Sickle Cell Trait (HbAS), the patient is heterozygous. Because the normal beta-globin allele produces hemoglobin slightly more efficiently than the mutated allele, the amount of HbA is always greater than HbS.
• A typical HPLC for sickle cell trait shows about 60% HbA and 40% HbS.
• In the prompt, HbS is described as "very high" (implying it is the major fraction, $>$ 50%) while HbA is the minority at around 40%.
• Since HbS $>$ HbA, this completely rules out Sickle Cell Trait (Option B).
• In classic, un-transfused Sickle Cell Disease (HbSS, homozygous mutation), the patient produces absolutely no normal beta-globin. Therefore, their HPLC will show roughly 90-95% HbS, some HbF, normal or slightly elevated HbA2, and 0% HbA.
• How can a patient with HbSS have 40% HbA? This occurs when the patient has recently received a blood transfusion (usually an exchange transfusion for complications like acute chest syndrome or stroke prevention).
• The transfused normal donor blood introduces HbA into circulation.
• The target for chronic exchange transfusions in HbSS is typically to reduce the HbS fraction to below 30-50%, resulting in a significant presence of HbA (e.g., 40-60%).
• Given the options, this profile represents a transfused patient with Sickle cell disease (HbSS).
• Beta-thalassemia trait presents with elevated HbA2 ($>$ 3.5%) and microcytic anemia, not HbS.
• HbC disease involves Hemoglobin C, not Hemoglobin S.
Final answer:
The pattern of HbS being the dominant hemoglobin alongside 40% HbA strongly suggests transfused Sickle Cell Disease (HbSS).
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