Indications to begin chronic transfusion therapy in HbE β thalassemia
| Indications . |
|---|
| Hb <6 g/dL at baseline |
| Hb 6-8 g/dL accompanied by symptoms |
| • Growth |
| − Infants (<2 y): failure to gain weight for 3 mo |
| − Children: Height velocity <3 cm/y |
| − Older children: Delay in puberty: >12 y in females, >13 y in males, endocrine evaluation |
| • Skeletal facial changes: subjective, discuss with patient and family |
| • Splenomegaly: Spleen >6 cm, or enlargement >1 cm/y after 2 y of age |
| • Extramedullary hematopoiesis: symptomatic or moderate to large masses |
| • Cerebrovascular: overt stroke, silent infarcts, arterial narrowing, moyamoya |
| • Venous thromboembolism |
| • Pulmonary hypertension |
| • Osteoporotic fracture |
| • Quality of life in adults: decline in capacity to work or perform usual activities |
| Indications . |
|---|
| Hb <6 g/dL at baseline |
| Hb 6-8 g/dL accompanied by symptoms |
| • Growth |
| − Infants (<2 y): failure to gain weight for 3 mo |
| − Children: Height velocity <3 cm/y |
| − Older children: Delay in puberty: >12 y in females, >13 y in males, endocrine evaluation |
| • Skeletal facial changes: subjective, discuss with patient and family |
| • Splenomegaly: Spleen >6 cm, or enlargement >1 cm/y after 2 y of age |
| • Extramedullary hematopoiesis: symptomatic or moderate to large masses |
| • Cerebrovascular: overt stroke, silent infarcts, arterial narrowing, moyamoya |
| • Venous thromboembolism |
| • Pulmonary hypertension |
| • Osteoporotic fracture |
| • Quality of life in adults: decline in capacity to work or perform usual activities |