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Special Feature
December 15, 2008

Image of the Month—Diagnosis

Arch Surg. 2008;143(12):1232. doi:10.1001/archsurg.143.12.1232
Answer: Extramedullary Hemopoiesis

Extramedullary hemopoiesis is the result of a compensatory mechanism against many chronic hematological diseases that are characterized by bone marrow depression. These conditions include thalassemia major or intermedia, sickle cell disease, polycythemia vera, chronic myelogenous leukemia, and hereditary spherocytosis. The most common sites of occurrence are the liver, spleen, and lymph nodes,1but many tissues have the potential to exhibit extramedullary hemopoiesis, such as the kidney, adrenal glands, breasts, spinal cord, pleura, endometrium, intracranial cavity, and intrathoracic cavity. Intrathoracic extramedullary hemopoiesis is rarely seen and is usually asymptomatic, requiring no therapy.2However, it may sometimes present with spinal cord compression, pleural effusion, massive hemothorax, or dyspnea.3It is important to distinguish a mass caused by extramedullary hemopoiesis from other lesions involving the posterior mediastinum. Diagnosis is based on clinical and radiological findings, with magnetic resonance imaging as the diagnostic modality of choice.4The differential diagnosis includes neurogenic tumors, lymphomas, primary and secondary malignant lesions, extrapleural cysts, and paravertebral abscesses. Biopsy may sometimes be necessary to confirm the diagnosis,2and surgery and radiotherapy may be needed to treat symptomatic patients with spinal cord compression.

Although extramedullary hemopoiesis is a rare cause of an intrathoracic mass, the diagnosis must be considered in any patient with congenital hemolytic disorder. In this regard, a potentially invasive procedure such as surgery should be avoided.

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The Editor welcomes contributions to the Image of the Month. Manuscripts should be submitted via our online manuscript submission and review system (http://manuscripts.archsurg.com). Articles and photographs accepted will bear the contributor's name. Manuscript criteria and information are per the Instructions for Authors for Archives of Surgery (http://archsurg.ama-assn.org/misc/ifora.dtl). No abstract is needed, and the manuscript should be no more than 3 typewritten pages. There should be a brief introduction, 1 multiple-choice question with 4 possible answers, and the main text. No more than 2 photographs should be submitted. There is no charge for reproduction and printing of color illustrations.

Correspondence:Stylianos Germanos, MD, Nuffield Department of Surgery, John Radcliffe Hospital, Headley Way, Headington, Oxford OX3 9DU, England (stylianos.germanos@yahoo.co.uk).

Accepted for Publication:February 1, 2007.

Author Contributions:Study concept and design: Stavrothanasopoulou, Germanos, and Xipolitos. Acquisition of data: Stavrothanasopoulou, Xipolitos, Ntochas, Stathopoulou, and Demertzis. Analysis and interpretation of data: Stathopoulou, Demertzis, Agelidou, and Vlastos. Drafting of the manuscript: Germanos, Xipolitos, Stathopoulou, Demertzis, and Agelidou. Critical revision of the manuscript for important intellectual content: Stavrothanasopoulou, Germanos, Ntochas, and Vlastos. Study supervision: Germanos.

Financial Disclosure:None reported.

Bartlett  RPGreipp  PRTefferi  ACupps  REMullan  BPTrastek  VF Extramedullary hematopoiesis manifesting as a symptomatic pleural effusion.  Mayo Clin Proc 1995;70 (12) 1161- 1164PubMedArticle
Calhoun  SKMurphy  RCShariati  NJacir  NBergman  K Extramedullary hematopoiesis in a child with hereditary spherocytosis: an uncommon cause of an adrenal mass.  Pediatr Radiol 2001;31 (12) 879- 881PubMedArticle
Xiros  NEconomopoulos  TPapageorgiou  EMantzios  GRaptis  S Massive hemothorax due to intrathoracic extramedullary hematopoiesis in a patient with hereditary spherocytosis.  Ann Hematol 2001;80 (1) 38- 40PubMedArticle
Chourmouzi  DPistevou-Gompaki  KPlataniotis  GSkaragas  GPapadopoulos  LDrevelegas  A MRI findings of extramedullary haemopoiesis.  Eur Radiol 2001;11 (9) 1803- 1806PubMedArticle