Phlebotomy in beta-thalassemia intermedia and secondary hemosiderosis
Lionelllo Camba
Dept. of Hematology, Ospedale S. Raffaele, Milan, Italy
Correspondence: Lionello Camba, Dept. of Hematology, Ospedale S.
Raffaele, via Olgettina 60, 20132 Milan, Italy. email:
l.camba@hsr.it
In their case report, De Gobbi et al.1 treated a
patient with beta-thalassemia trait and juvenile hemochromatosis
using recombinant human erythropoietin to stimulate
erythropoiesis, iron mobilization, prevent anemia and allow
frequent phlebotomies. A low Hb need not always deter from regular
venesections when thalassemia is associated with increased iron
deposition, like in the case described below of a patient with
thalassemia intermedia and secondary hemosiderosis. A 52-yr old
man presented with severe and prolonged hematuria due to
hemorrhagic cistitis and fever. A diagnosis of beta thalassemia
with high HbF and jaundice with splenomegaly was made 30 yrs
earlier. His past Hb levels were consistently below 105 g/L. On
admission he was jaundiced; liver and spleen were palpable 5 cm
below the costal margins. Blood tests: Hb 63 g/L, MCV 72 fl,
leukocytes 3.2x109/L with normal differential count;
total bilirubin 70 mmol/L, unconjugated bilirubin 66 mmol/L;
ferritin 795 mg/L, serum iron 12 mmol/L (12-30), TIBC 32 mmol/L,
transferrin saturation 38% (both infection and bleeding must have
changed the expected pattern of iron overload); low haptoglobins;
negative direct Coombs test; elevated HbA2 (4.1%) and HbF (10%);
normal liver enzymes and kidney function. Antibodies anti HCV were
positive. Bone marrow aspirate showed marked erythroid hyperplasia
and increase of macrophage iron. Abdominal ultrasound scan showed
hepato-splenomegaly, gallstones and blood clots in the bladder.
Following antibiotics and blood trasfusion the patient improved
and was discharged home two weeks later. Three months later his Hb
was 100 g/L, ferritin 1060 mg/L, serum iron 39 mmol/L, transferrin
1.82 g/L (2.0-3.3). He was otherwise asymptomtic. Liver and
thyroid functions remained normal. Glucose tolerance test was
slightly impaired, with normal HbA1C. To prevent further iron
deposition, and considering his HCV status, once/wk venesections
of 100 ml and slow i.v. desferrioxamine (2 g and then 1 g after
six months) the day of phlebotomy were started. Ferritin levels
fell gradually over the following months to a nadir of 249 mg/L,
the Hb never dropped below 95 g/L; the patient could hold his job
as a teacher and live a normal life. Treatment was withdrawn after
18 months when the patient was admitted for cholecystectomy due to
obstructive jaundice. Gallstones were of mixed
cholesterol/bilirubin type. The patient has now been off treatment
for 5 years. As of April 2000 ferritin, transferrin and serum iron
were respectively 349 mg/L, 25 mmol/L, and 1.9 g/L, Hb 102 g/L,
liver enzymes and HbA1C normal. This case illustrates that
patients with mild thalassemia intermedia and secondary
hemosiderosis can tolerate weekly small-volume phlebotomies with
little or no interference with their quality of life, their
performance status and without the costs of rHuEPO. Compliant
patients may reach a negative iron balance more efficiently if
more intense iron chelation is implemented. Our approach cannot,
of course, apply to transfusion dependent patients or when a
negative balance is to be achieved quickly and removal of large
volumes of blood is needed, as in De Gobbi et al's report, where
the thalassemia phenotype caused a profound drop of Hb following
phlebotomy.
