12th Congress of the European Hematology ... - Haematologica

p=0.014 and p=0.003, respectively). In the totality of patients, myocardial
T2* values were inversely correlated to age (r=-0.249, p=0.024) and
positively correlated to both left and right ventricular ejection fraction
(r=0.33, p=0.004 and r=0.279, p=0.014, respectively). Liver T2* was
strongly inversely correlated with serum ferritin concentrations (r=0.465,
p=0.001). Finally, no correlation was noted between myocardial and
hepatic T2* values (r=-0.043, p=0.37). Conclusions. Our results indicated
that DFP is more effective than DFO in removal myocardial iron, whereas
DFO favours in removing hepatic iron compared to DFP. Combined
chelation treatment with DFP and DFO seems to better control both
myocardial and hepatic iron.
0804
HIGH DEGREE OF IRON BURDEN IN TRANSFUSION-NAIVE THALASSEMIA INTERMEDIA
PATIENTS
F. Rassi, 1 H. Ismaeel, 1 A. Inati, 2 S. Koussa, 3 D. Habr, 4 A. Taher1 1 American University of Beirut, BEIRUT; 2 Nini Hospital, TRIPOLI; 3 Chronic
Care Center, HAZMIEH; 4 Novartis Pharma Services, BEIRUT, Lebanon
Background. Iron accumulation in Thalassemia intermedia (TI) patients
has been estimated to occur at a rate of 2-5 gm/yr and occurs mostly as
a result of increased gut iron absorption secondary to chronic anemia.
Increased iron burden is partly responsible for the occurrence of complications
that occur in this disease such as liver fibrosis, heart failure and
pulmonary hypertension; therefore, close and accurate monitoring of
total body iron is necessary for ultimate proper chelation therapy. To this
day, serum ferritin (SF) has been the main tool used to follow up estimated
iron overload. However the inaccuracy of this test due to confounding
factors and its poor correlation with biopsy-determined liver iron
concentration (LIC) (the gold standard) has prompted researchers to develop
other non-invasive techniques such as R2 MRI which has recently
gained FDA approval. The poor correlation between SF level increase and
LIC has been shown to be particularly true in patients with TI. Aim. To
determine the extent of iron overload in 80 randomly selected TI patients
in Lebanon. Methods. Following informed consent and extensive relevant
medical history review, LIC using R2 MRI, SF, and other Iron markers
are measured. Also Doppler echocardiography is done to detect pulmonary
hypertension and left ventricular ejection fraction. This abstract
reports results for the sub-group of patients who have never received any
transfusion in their lifetime. Results. Thirty-seven completely transfusion-naïve
patients are included in this analysis. This subgroup comprised
16 males and 21 females with a mean age of 27.5 years (range 8
to 63 yrs). Of these patients, 27 were splenectomized and 10 were not.
Overall LIC values ranged from 0.6 to 32.10 mg Fe/g of dry tissue liver
with a mean LIC of 7.76±6.78. SF values ranged from 19 to 2030 ng/ml
with a mean of 957.35±553.09 ng/mL. Overall, there a significant positive
correlation between LIC and SF values (p-value 0.014) and this correlation
was particularly true for the subset of splenectomized patients
(p-value 0.005). Statistical analysis also revealed significant correlations
between LIC and splenectomy on one side and SF and splenectomy on
the other (p-value 0.05 and 0.009 respectively). In addition, echocardiographic
analysis of the population has revealed a tricuspid regurgitant
(TR) jet in 59.5% of the studied patients. The detection of TR represents
a step toward the development of pulmonary hypertension as a
complication. Other complications included one case of thrombosis, 2
cases of extramedullary hematopoiesis and leg ulcers, 3 patients had
hypothyroidism and 14 had osteoporosis. Conclusions. Although nontransfused,
this group of TI patients has evidence of significant iron burden
as evident by the increased mean LIC and SF. There was good correlation
between LIC and SF in splenectomized transfusion-naïve TI.
This is contrary to previous reports that showed discrepancy between
LIC and SF in the same population. Our analysis will gain more power
as we enroll all 80 patients in the study in the coming few months. In
addition, we will be able to extrapolate our results further and determine
the need to address the issue of chelation therapy in this iron overloaded
population.
12 th Congress of the European Hematology Association
0805
NEUTROPENIA AND AGRANULOCYTOSIS IN IRON OVERLOADED THALASSEMIA MAJOR
PATIENTS, TREATED WITH COMBINATION OF DEFERRIOXAMINE AND DEFERIPRONE
M. Hadjigavriel, 1 M. Sitarou, 2 A. Kolnacou, 3 E. Pangalou, 4
E.R. Savvidou, 5 S. Cristou4 1 Limasssol General Hospital, LIMASSOL; 2 Larnaca General Hospital, LAR-
NACA; 3 Pafos General Hospital, PAFOS; 4 Makarios Hospital, NICOSIA;
5 Nicosia General Hospital, NICOSIA, Cyprus
Background. Combination chelating therapy with Deferrioxamine
(DFO) and Deferiprone (L1) has a specific protective effect mainly
because adherence to treatment is improved and because L1 has a cardioprotective
effect in iron overloaded thalassemia patients. Neutropenia
and agranulocytosis are the most important side effects during L1
monotherapy. Neutropenia was also described in patients treated with
DFO. Some reports showed that the frequency of these effects is higher
with combination treatment, than with either drug alone. Aims. The
aim of this study was to evaluate the frequency and incidence rate of
neutropenia and agranulocytosis over a period of 6 years in patients
receiving combination treatment. Methods. A total of 345 regularly transfused
thalassemia major patients, from 4 thalassemia centers were
assessed representing treatment with DFO/L1 from 3 months to 6 years.
Patients started combination with L1 at a dose of 75 mg/kg daily divided
into three doses, together with DFO 30-50 mg/kg/2-5 times/week
mainly on s.c infusion. Assessment of the blood count with differential
was performed every 7-10 days. A patient was considered to have neutropenia
if the neutrophil count was confirmed to be