absence of hepatomegaly (enlargeed liver), regular iron chelation
before transplant, absence of fibrosis in pretransplant liver
biopsy result
96%
90%
II
hepatomegaly, a history of irregular iron chelation
before transplant, histological evidence of liver fibrosis, or various
combinations of the above
86%
82%
III
all of the following: large liver, poor compliance
with chelation therapy, liver damage
76%
53%
Adult
Class II or III, irregular iron chelation, with a range of clinical
symptoms and other diagnoses
65%
63%
Bone Marrow Transplant (3)
32% of adults expired as a direct result of complications
of the bone marrow transplantation. Recently the Lucarelli group reported
on bone marrow recipients who have "mixed chimerism," meaning
these individuals have both their own thalassemic blood cells and donor
blood cells. In these cases about one third each either rejected the transplant,
totally engrafted the donor cells or remained a mixed chimera. The latter
two groups required no blood transfusions. It should be noted that the
outcomes at the Pesaro Centre are better than any other center reporting
bone marrow transplant results for beta thalassemia major. This may be
in part due to the homogeneity of the population.