Another common comorbidity in patients with beta thalassemia is bone disease, such as osteoporosis or osteopenia, which is associated with pain and functional limitations that can significantly affect patients’ quality of life. The underlying cause of bone disease is multifactorial, and vitamin D or calcium supplementation and bisphosphonate therapy are often prescribed for this indication. In some cases, denosumab or teriparatide may be prescribed for select patients.

“Bone disease is exceedingly common, even for patients in their 20s and 30s, and most [patients] are followed by an osteoporosis specialist and are on bisphosphonate therapy,” said Dr Merkeley.

Similar to the general population, an increase in the incidence of cancer has been reported in aging patients with beta thalassemia. The most common type of cancer observed in this population is hepatocellular carcinoma, as these patients experience a high frequency of known risk factors, including liver iron overload and hepatitis B and C infection. However, treatment for hepatocellular carcinoma, as well as for other tumor types and hematologic malignancies, should be the same as that administered to the general population, and the presence of comorbidities and performance status ought to be taken into consideration just as for any other patient.


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Dr Merkeley noted that in her practice, she has only seen 1 patient who was diagnosed with a malignancy; the patient had thyroid cancer while in her 30s. “I recommend age-appropriate malignancy screening for all [of] my patients, including Pap smears, mammograms, colonoscopies, and prostate cancer screening,” she said.

Finally, renal function needs to be monitored in patients with beta thalassemia, although the researchers highlighted that data on kidney disease in thalassemia are scarce and have primarily come from small, single-center, cross-sectional, observational investigations. One study has reported the presence of proximal tubular damage in patients with TDT, with low-molecular-weight proteinuria present in nearly the entire cohort. Abnormalities in glomerular filtration rates have also been observed in patients with beta thalassemia, as has hyperfiltration, which is related to anemia. The presence of these abnormalities can potentially lead to progressive decline in glomerular filtration rate over time.

“Renal disease is a big concern as patients get older because they are more likely to develop toxicity to our preferred — for most circumstances — chelator, deferasirox, if they have underlying renal impairment,” said Dr Merkeley. “If they are unable to take deferasirox, which is [administered] once daily, then they have to take a [3 times daily] oral chelator, deferiprone, or subcutaneous [deferoxamine], which patients hate and leads to decreased compliance.”

Complications can be more difficult to manage in this population because patients are so “medicalized” from such a young age that they generally try and avoid the hospital as much as possible, Dr Merkeley explained. “Therefore, for many of my patients, trying to get them to start new medications or see new specialists can be challenging, especially if it is something [for] which they are relatively asymptomatic.”

Physicians treating this population need to be more aware of these complications, especially as patients age, due to the complexity of chronic disease in the aging population. Dr Merkeley pointed out that with other conditions she treats, such as sickle cell anemia and hemophilia, she involves colleagues from the geriatric department. “None of my transfused thalassemia patients are at an age where I have had to involve geriatrics, but I anticipate that becoming more common,” she added.

Reference

1.     Motta I, Mancarella M, Marcon A, Vicenzi M, Cappellini MD. Management of age-associated medical complications in patients with β-thalassemia. Expert Rev Hematol. 202;13:85-94.