13 Mar

Massive Hemothorax Due to Intrathoracic Extramedullary Hematopoiesis in a Patient With Thalassemia Intermedia: Conclusion

Bone erosion and sclerosis occur in about 50 percent of patients with neurogenic tumors and lobulation is not seen. In addition, enhanced visualization of the masses on postcontrast computerized tomography supports the diagnosis of intrathoracic extra-medullary hematopoiesis as demonstrated in our case. Although invasive diagnostic procedures, including thoracotomy and aspiration needle biopsy, have been used, they are usually not necessary and are potentially hazardous because of the highly vascular nature of the thoracic masses. At thorocotomy Andras et al found the blood supply to derive from intercostal arteries and the esophageal plexus. Venous drainage was into the azygous system. In several reports surgical resection has been complicated by extensive hemorrhage requiring multiple transfusions. Our case provides a dramatic example of the hemorrhagic potential of this condition.

Treatment is required only in the presence of complications. Treatment may in fact be deleterious due to removal of a compensatory source of erythropoiesis and exacerbation of the underlying anemia. Extramedullary hematopoietic tissue is highly radiosensitive, and relatively small doses of radiation have been effective after surgical decompression in cases of spinal cord compression. In our patient radiotherapy was administered to prevent recurrent hemorrhage after surgical control of the acute bleeding.
It is unclear whether there was some factor in our patient which provoked the hemorrhage resulting in massive hemothorax. The lacerations in the capsule of the larger mass seen at thoracotomy may have resulted from spontaneous rupture. The role of the apparently superficial trauma that she sustained prior to admission remains speculative. Awareness of this potential complication of intrathoracic extramedullary hematopoiesis may make it possible to treat future cases with radiation and chest tube drainage without resorting to thoracotomy.

Categories: Thalassemia Intermedia
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