25 Feb

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

Massive Hemothorax Due to Intrathoracic Extramedullary Hematopoiesis in a Patient With Thalassemia IntermediaMassive extramedullary erythropoeisis is an unusual phenomenon, most frequently observed as a response to the intense intramedullary hemolysis associated with the homozygous β-thalassemias and related conditions, such as hemoglobin E-thalassemia. The subset of such patients who do not require transfusion (thalassemia intermedia, or mild thalassemia) is most likely to display this phenomenon, since their erythropoeisis is not suppressed. The erythropoietic masses are usually asymptomatic, although spinal cord compression has been reported. We describe a patient with thalassemia intermedia in whom hemorrhage from a large erythroid mass resulted in massive hemothorax, to our knowledge a heretofore unreported occurrence.

Case Report
The patient is a 46-year-old black woman with thalassemia intermedia. Hematologic and globin synthetic data concerning the patient and her family have been reported. Aside from severe anemia associated with her only pregnancy at age 21, and again with secondary hypersplenism at age 35 (successfully alleviated by splenectomy), her hemoglobin has been stable at about 8.0 g/dl. She has had long-standing hepatomegaly with mild abnormalities of liver chemistry studies. A liver biopsy specimen at age 40 showed hemochromatosis with slight fibrosis. At age 44, iron unloading with continuous, subcutaneous desferrioxamine infusion was initiated and has continued to date. She remained actively employed and clinically well.

She was long known to have a left posterior mediastinal mass on chest roentgenograms, which has gradually increased in size over the past 11 years (Fig 1) but produced no symptoms. A mass in the cul-de-sac had also been noted, and both were assumed to be extramedullary erythropoietic tissue. Extensive osteopenia, consistent with expansion of the hematopoietic marrow, had also been present for many years.

Figure 1A (upper). Chest roentgenogram in 1975 showing para-spinal mass due to extramedullary hematopoiesis (arrow). B (lower). Chest roentgenogram in 1986, two months before development of left hemothorax. Mass has enlarged progressively since 1975 (arrow).

Figure 1A (upper). Chest roentgenogram in 1975 showing para-spinal mass due to extramedullary hematopoiesis (arrow). B (lower). Chest roentgenogram in 1986, two months before development of left hemothorax. Mass has enlarged progressively since 1975 (arrow).

Categories: Thalassemia Intermedia
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