Περίληψη:
Background. Thalassemia intermedia has a later clinical onset and a
milder anemia than thalassemia major, characterized by high output
state, left ventricle remodeling, and age-related pulmonary
hypertension. Bone deformities, extramedullary hematopoiesis (EMH), and
spleen and liver enlargement are the consequences of hypoxia and
enhanced erythropoiesis. The EMH-related pleural effusion is rarely
referred to in the literature of thalassemia.
Methods. We reviewed the thalassemia patients’ medical records
hospitalized for pleural effusion in our Department, within the last 6
years.
Results. Eight ( 4 men) thalassemia intermedia patients admitted for
symptomatic pleural effusion were identified. Common clinical findings
on admission were dyspnea and apyrexia. Their mean hemoglobin level was
7.15 +/- 0.64 g/dL. Radiology revealed intrathoracic EMH and pleural
effusion in all patients: exudative in seven patients and massive
hemothorax in one. Cytologic fluid analysis was negative for malignancy.
Fluid and serum cultures, antibodies, and stains were negative for
viral, bacterial, and fungal infection. The hemothorax case was
successfully treated with repeated aspirations, transfusions, and
hydroxyurea. Although repeated thoracentesis and radiation could not
control the effusions in the rest of the cases, pleurodesis was
successful in 5 patients, without serious adverse events. Treatment was
further accomplished with hydroxyurea. No relapses were observed in the
mean 30 month follow-up period.
Conclusions. Afebrile, EMH-related pleuritis represents a potentially
life-threatening complication in thalassemia. Therapy should be
individualized and treatment is emerging. Pleurodesis seems to be an
effective and safe therapeutic option for exudative effusions, while
transfusion-chelation therapy combined with hydroxyurea may be helpful
in suppressing increased erythropoiesis.
Συγγραφείς:
Aessopos, Athanassios
Tassiopoulos, Stergios
Farmakis, Dimitrios
and Moyssakis, Ioannis
Kati, Maria
Polonifi, Katerina and
Tsironi, Maria
