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How I manage medical complications of β-thalassemia in adults

Ali T. Taher and Maria Domenica Cappellini

Article Figures & Data

Figures

  • Figure 1.

    Management priorities across age groups in TDT. The listing reflects potential incidence of morbidities in respective age groups, but is not exclusive, and individual patients may have different needs. aIncludes diabetes mellitus, hypothyroidism, hypoparathyroidism, osteoporosis, hypogonadism.

  • Figure 2.

    General monitoring recommendations across age groups in TDT. aBy MRI R2 for liver or MRI T2* for liver and heart with appropriate calibration. LIC and cardiac T2* may be assessed at earlier age (from 6 years) if feasible especially in patients who are on high iron intake. bAs assessed by experienced echocardiographer or cardiac MRI. cAlanine aminotransferase, aspartate aminotransferase, total and direct bilirubin. dThyroid-stimulating hormone; calcium, phosphate, vitamin D, and parathyroid hormone (as indicated); luteinizing hormone, follicle-stimulating hormone, testosterone, estradiol, gonadotropin-releasing hormone (as indicated in cases of abnormal sexual development); fasting blood sugar, oral glucose tolerance test (as indicated). BMD, bone mineral density; ECG, electrocardiogram; LIC, liver iron concentration; LVEF, left-ventricular ejection fraction; Q, every; TE, transient elastography; TRV, tricuspid-regurgitant jet velocity; ULN, upper limit of normal; US, ultrasound.

  • Figure 3.

    Morbidities and risk factors in patients with NTDT. Risk factors and pathophysiologic mechanisms as Venn diagrams within which notable morbidities associated with them are included. Some morbidities are attributed to >1 risk factor. These associations are mostly based on data from observational studies. aAs evident on fluorodeoxyglucose positron emission tomography–computed tomography (PET-CT). EMH, extramedullary hematopoietic pseudotumors; GFR, glomerular filtration rate; IE, ineffective erythropoiesis, PHT, pulmonary hypertension.

  • Figure 4.

    Approach to monitoring and management of liver disease in adult β-thalassemia patients. aBy MRI R2 or T2*, the latter requiring appropriate calibration and preferred if cardiac iron assessment is required at the same time. MRI monitoring can start at the age of 10 years in TDT (or earlier if feasible and deemed necessary) and NTDT. bIf not anti-HBsAg+. DAA, direct-acting antiviral drug; HAV, hepatitis A virus; HBV, hepatitis B virus; HCV, hepatitis C virus; LFT, liver function test; LIC, liver iron concentration; RNA-PCR, RNA polymerase chain reaction; SF, serum ferritin.

  • Figure 5.

    Approach to diagnosis, prevention, and management of pulmonary hypertension in adult patients with β-thalassemia. aPatients with TRV < 2.5 m/s may also be reassessed but at longer intervals (3-5 years). nRBC, nucleated red blood cell count; PHT, pulmonary hypertension.