Chest
Volume 103, Issue 3, March 1993, Pages 974-975
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Selected Reports
Massive Hemothorax Associated with Intrathoracic Extramedullary Hematopoiesis Involving the Pleura

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Intrathoracic extramedullary hematopoiesis rarely involves the pleura and is usually asymptomatic. We report a 73-year-old woman with myelofibrosis who had pleural involvement with extramedullary hematopoietic tissue that produced a massive hemothorax. Before the diagnosis of extramedullary hematopoietic tissue was established, sclerosis with tetracycline was attempted, which accelerated pleural bleeding and required surgical evacuation. The bleeding was ultimately controlled by low-dose radiation therapy.

Section snippets

CASE REPORT

A 73-year-old woman with a history of myelofibrosis diagnosed 7 years earlier presented with dyspnea and a right-sided pleural effusion. Sanguineous fluid (1,000 ml) was removed, after which her symptoms improved. The pleural fluid was an exudate and had an RBC of 0.13 × 1012VL, a WBC of 5.5 × 109/L (63 percent neutrophils, 11 percent lymphocytes, 7 percent eosinophils, 3 percent metamyelocytes, 9 percent myelocytes, and 4 percent promyelocytes), and a hematocrit reading of less than 2 percent.

DISCUSSION

Intrathoracic extramedullary hematopoiesis is typically asymptomatic unless it causes symptoms from compression of nearby structures, such as the spinal cord.2, 5 In our patient, the extramedullary hematopoietic tissue involved the pleura, resulting in a massive hemothorax. Pleural extramedullary hematopoiesis in myelofibrosis has been described previously,4 but these patients did not have respiratory symptoms, and the diagnosis was made at autopsy. In one other case report,6 massive hemothorax

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    The most common sites of EMH are liver and spleen, but it has been documented in other organs such as the mediastinum, lymph nodes, breast, and central nervous system [1]. Among them, intrathoracic EMH is commonly encountered as a mass in the posteroinferior mediastinum, though there have been a few reports of the anterior mediastinum or pleura [2]. In patients with a medical history of neoplastic or hematologic disorders presenting with intrathoracic mass, a diagnosis of EMH should be considered in the differential diagnosis.

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    Chute et al reported the first fatal hemothorax from spontaneous rupture of intrathoracic EMH in 2004.4 Kupferschmid et al reported a case of myelofibrosis with intrathoracic EMH.5 This complicated massive hemothorax did not respond to tetracycline pleurodesis and was ultimately controlled with low-dose radiation therapy.

  • Intrathoracic Extramedullary Haematopoiesis Manifested as a Neoplastic Lesion Within Anterior Mediastinum

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    Intrathoracic EMH, the formation of appearently normal blood cells outside the confines of the bone marrow is uncommon, but is a well-defined entity [1]. It is usually asymptomatic and located in the lower paravertebral sulci or rarely in the pleura [2]. If hematologic disorder and radiologic evidence of myelofibrosis are present, EMH is easy to diagnose.

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†Presently at the Department of Thoracic and Cardiovascular Surgery, University Hospital, Boston.

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