(Chest. 2003;124:732-734.)
© 2003
American College of Chest Physicians
A Patient With Anemia and a Paraspinal Chest Mass*
Chandana Lall, MD and
D. Keith Payne, MD, FCCP
* From the Departments of Radiology (Dr. Lall) and Pulmonary and Critical Care Medicine (Dr. Payne), Louisiana State University Health Sciences Center and the Feist Weiller Cancer Center, Shreveport, LA.
Correspondence to: D. Keith Payne, MD, FCCP, Professor of Medicine, Division of Pulmonary & Critical Care Medicine, LSU Health Sciences Center-Shreveport, 1501 King’s Hwy, Shreveport, LA 71130; e-mail: kpayne{at}lsuhsc.edu
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Introduction
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A 56-year-old African-American man presented to the emergency department with mild chest pain and otalgia. He also noted a nonproductive cough of several days’ duration and mild back pain. He denied the presence of any fever, chills, or hemoptysis. His medical history was remarkable for homozygous sickle cell disease with multiple complications, including a cerebrovascular accident in September 1999 with resultant hemiparesis as well as peptic ulcer disease. The patient had a long history of hypertension, which was controlled by medication. He reported having undergone multiple blood transfusions, but none in the last 2 years. He denied any recent sickle cell crises.
A physical examination revealed a well-nourished, afebrile black man who was in no acute distress. His hospital admission BP was 183/93 mm Hg, and his respiratory rate was 18 breaths/min with a pulse rate of 83 beats/min. A cardiovascular examination demonstrated a systolic murmur 3/6 at the upper left sternal border. Bibasilar and midlung crackles were noted. Laboratory studies showed normal levels of electrolytes, BUN, and creatinine, with a hemoglobin level of 7.8 g/dL and hematocrit of 24.9%. Prothrombin time and activated partial thromboplastin time levels were in the normal range. A chest radiograph obtained after hospital admission revealed cardiac enlargement with a mildly prominent interstitial pattern in the lungs. A left paraspinal mass also was noted (Fig 1
), prompting a CT scan of the chest (Fig 2
).
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What is the diagnosis?
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Diagnosis: Extramedullary hematopoiesis secondary to sickle cell disease
The presence of well-defined unilateral or bilateral paraspinal masses and/or paracostal masses in patients with homozygous sickle cell disease should alert one to the presence of extramedullary hematopoiesis.1
Extramedullary hematopoiesis is a rare cause of posterior mediastinal masses and is usually seen in patients with severe, long-standing anemia. This represents an abnormal expansion of erythropoietic tissue and results in multiple paraspinal masses and hypertrophy of the medullary cavity of the ribs. This entity is commonly seen in patients with thalassemia but may also be encountered in those with sickle cell disease, hereditary spherocytosis, myelosclerosis, and other causes of anemia.2
3
A rare case of extramedullary hematopoiesis in a patient with alcohol-related macrocytosis, which was detected using 99mTc sulfur colloid scintigraphy of the lower chest and abdomen has been reported.4
The plain radiographic manifestations of thoracic extramedullary hematopoiesis are unilateral or bilateral, smooth, lobulated masses without erosion of the vertebral bodies or ribs, sometimes associated with subpleural paracostal masses. CT scanning shows well-circumscribed, smooth, soft tissue attenuation masses, usually at multiple levels in a paraspinal location without erosion or pressure changes on the adjacent ribs or vertebral bodies, in contrast to paraspinal neurofibromas, which usually have associated osseous changes. Older inactive masses may reveal iron deposition and/or fatty replacement.5
Radionuclide scanning using colloids also has been useful in detecting extramedullary hematopoiesis. This technique depends on the ability of reticuloendothelial cells to transform colloid particles through phagocytosis and frequently demonstrates marrow expansion in the extremities as well as uptake in other areas, such as the paraspinal region.6
Most patients are asymptomatic, and, since these masses tend to be slow-growing, patients should not be subjected to aggressive diagnostic and therapeutic measures.1
Rarely, very significant enlargement of paraspinal hematopoietic masses has been known to produce cord compression.7
Nerve root compression with radicular pain also has been reported.3
A rare case of a 49-year-old woman with thalassemia intermedia who developed a massive hemothorax from a large thoracic paraspinal hematopoietic mass has been reported.8
A case of symptomatic extramedullary hematopoiesis in an adult man with thalassemia showed the regression of paraspinal masses after treatment with hydroxyurea.9
In severe cases, irradiation has been performed.7
Received for publication August 8, 2001.
Accepted for publication March 18, 2002.
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References
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- Gumbs, RV, Higginbotham-Ford, EA, et al (1987) Thoracic extramedullary hematopoiesis in sickle cell disease. AJR Am J Roentgenol 149,889-893[Abstract/Free Full Text]
- Vlahos, L, Trakadas, S, Gouliamos, A Retrocrural masses of EMH in beta thalassemia. Magn Reson Imaging 1993;11,1227-1229[CrossRef][ISI][Medline]
- Gouliamos, A, Dardoufas, C, Papailiou, I, et al Low back pain due to extramedullary hemopoiesis. Neuroradiology 1991;33,284-285[CrossRef][ISI][Medline]
- De Geeter, F, Van Renterghem, D Scintigraphic diagnosis of intrathoracic extramedullary hematopoiesis in alcohol related macrocytosis. J Nucl Med 1996;37,473-475[Abstract/Free Full Text]
- Tsitouradis, J, Stamos, S, Hassapopoulou, E, et al Extramedullary paraspinal hematopoiesis in thalassemia: CT and MRI evaluation. Eur J Radiol 1998;30,33-38[CrossRef][ISI]
- Bronn, LJ, Paquelet, JR, Tetalman, MR Intrathoracic extramedullary hematopoiesis: appearance on 99mTc sulfur colloid marrow scan. Am J Radiol. 1980;134,1254-1255[ISI][Medline]
- Mehta, J, Singhal, S, Sampat, NG Extramedullary hematopoietic mass with cord compression after splenectomy in thalassaemia and response to radiation. J Assoc Physicians India 1995;43,563-564[Medline]
- Smith, PR, Manjoney, DL, Teitcher, JB, et al Massive hemothorax due to intrathoracic extramedullary hematopoiesis in a patient with thalassemia intermedia. Chest 1998;94,658-660[CrossRef]
- Saxon, BR, Rees, D, Olivieri, NF Regression of extramedullary haemopoiesis and augmentation of fetal hemoglobin concentration during hydroxyurea therapy in beta thalassaemia. Br J Haematol 1998;101,416-419[CrossRef][ISI][Medline]
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Introduction
What is the diagnosis?
