Bilirubin Levels in Beta-Thalassemia: Causes and Concerns - Oncology

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Bilirubin in Mediterranean Anemia


Hello, Doctor: I have been diagnosed with Beta-thalassemia.
In previous follow-ups, my hemoglobin levels were around 9.3 g/dL, red blood cell count was 4.5 M/uL, total bilirubin was approximately 6.0 mg/dL, and direct bilirubin was 0.4 mg/dL.
In this year's health check-up, I found that my hemoglobin dropped to 8.0 g/dL, red blood cell count to 3.8 M/uL, total bilirubin to 7.7 mg/dL, and direct bilirubin remained at 0.4 mg/dL.

I have the following questions:
1.
Does this indicate that both the anemia and hemolysis have worsened? Does the severity of thalassemia change with aging, or could there be other reasons for the worsening anemia?
2.
Since my direct bilirubin level is still within normal range and other liver function tests are normal, is the elevated total bilirubin solely due to hemolysis? For many years, it has been attributed to anemia, but I notice that thalassemia patients typically do not have such high bilirubin levels.
Could there be other causes, and what tests should I consider?
3.
With bilirubin levels that high, aside from causing jaundice, could leaving it untreated lead to serious consequences?

FU, 60~69 year old female. Ask Date: 2021/09/28

Dr. Chen Yunfang reply Oncology


Hello:
1.
Yes, both anemia and hemolysis have worsened.
However, this congenital thalassemia should not change with aging, so there must be something that has happened recently.
Have you had a cold? Taken medications indiscriminately? Used traditional Chinese medicine? Consumed health foods? Experiencing excessive stress? There are many possible exacerbating factors, so think about it.

2.
You are correct that other individuals with thalassemia do not exhibit as much jaundice; most people have mild symptoms and can even serve in the military without issues.
Your values suggest moderate to severe thalassemia, which is quite rare.

3.
It is common to experience itchy skin, and medications or topical treatments may not be effective.
As for more severe effects, I have not encountered them.

Thank you.

Reply Date: 2021/09/28

More Info


Understanding bilirubin levels in patients with beta-thalassemia is crucial, especially when there are changes in hemoglobin and bilirubin levels over time. In your case, you have noted a decrease in hemoglobin from approximately 9.3 g/dL to 8.0 g/dL, alongside a rise in total bilirubin from 6.0 mg/dL to 7.7 mg/dL, while direct bilirubin remains stable at 0.4 mg/dL. Let's address your questions systematically.

1. Severity of Anemia and Hemolysis: The decrease in hemoglobin and red blood cell count suggests that your anemia may indeed be worsening. In beta-thalassemia, the severity of anemia can fluctuate due to various factors, including nutritional deficiencies (such as iron, vitamin B12, or folate), infections, or even splenic function. The increase in total bilirubin levels, particularly in the context of beta-thalassemia, often indicates increased hemolysis (destruction of red blood cells). In patients with beta-thalassemia, the body compensates for the ineffective erythropoiesis (production of red blood cells) by increasing red blood cell turnover, which can lead to elevated bilirubin levels. It is essential to monitor these parameters regularly, as changes can indicate a need for further intervention or treatment.

2. Causes of Elevated Total Bilirubin: Given that your direct bilirubin level is within normal limits and other liver function tests are normal, it is reasonable to conclude that the elevated total bilirubin is primarily due to hemolysis associated with your beta-thalassemia. However, it is important to consider other potential causes of elevated bilirubin, such as liver dysfunction, biliary obstruction, or other hemolytic conditions. Since you mentioned that other patients with beta-thalassemia do not typically exhibit such high bilirubin levels, it may be prudent to investigate further. Additional tests could include a complete blood count (CBC) with reticulocyte count, peripheral blood smear, haptoglobin levels, and possibly a direct Coombs test to rule out autoimmune hemolytic anemia.

3. Consequences of Elevated Bilirubin: While mild elevations in bilirubin can lead to jaundice (yellowing of the skin and eyes), the primary concern with significantly elevated bilirubin levels is the potential for bilirubin toxicity, particularly in cases of severe hyperbilirubinemia. Chronic high levels of bilirubin can lead to complications such as kernicterus in newborns, but in adults, the risks are generally lower. However, prolonged elevations can indicate underlying issues that may require treatment. It is essential to address the root cause of the elevated bilirubin rather than merely treating the symptom of jaundice.
In summary, your situation warrants close monitoring and possibly further investigation to determine the underlying causes of your worsening anemia and elevated bilirubin levels. Regular follow-ups with your healthcare provider are essential to manage your beta-thalassemia effectively and to address any complications that may arise. If you notice any new symptoms, such as increased fatigue, abdominal pain, or changes in urine or stool color, it is crucial to seek medical attention promptly.

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