TABLE 2.

Criteria for initiation of regular transfusions

1. Hemoglobin <7 g/dl on 2 occasions at least 2 weeks apart

a. β thalassemia major: <7 g/dl on 2 occasions, with or without symptoms

b. HbE β thalassemia: <7 g/dl on 2 occasions and one or more of the symptoms listed in Section 3

2. Hemoglobin ≥7 g/dl, with one or more of the following symptoms

a. Growth delay:

i. Infants (<2 years): failure to gain weight for 3 months without another etiology

ii. Children: Height velocity <3 cm/year

iii. Delayed onset of puberty: >12 years in females, >13 years in males, with endocrine evaluation

b. Skeletal facial changes: subjective, photographic record, discuss with patient and family

c. Splenomegaly: Spleen >6 cm, or enlargement >1 cm/year after 2 years of age

d. Extra‐medullary hematopoiesis: symptomatic or moderate to severe EMH

e. Cerebrovascular: overt stroke, silent infarcts, arterial narrowing, moya moya

f. Venous thrombo‐embolism

g. Pulmonary hypertension

h. Osteoporotic fracture

i. Poor quality of life in adults: decline in capacity to work or perform usual activities