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Cases
Fatigue and Abdominal Fullness
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Background
A young man presents to the hospital with an insidious onset of fatigue, chest tightness, and abdominal fullness. Chest radiographs and abdominal CT scans are obtained.
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Scroll
down for Hint and Answer
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***** HINT *****
The patient has a chronic, systemic process.
***** ANSWER *****
Extramedullary hematopoiesis in beta thalassemia:
As seen on the posteroanterior (Image A) and
lateral (Image B) chest radiographs, the patient
has multiple, large posterior mediastinal masses.
Splenomegaly is present on the abdominal CT scout
image (Image C). Cholecystectomy clips are also
present. On the first chest CT scan (Image D),
the posterior mediastinal masses have soft tissue
attenuation but do not involve the neural foramina.
On the second CT scan (Image E), the vertebral body
and ribs are sclerotic, with coarse trabeculae.
Osseous sclerosis and splenomegaly can be seen in
combination in several entities (eg, mastocytosis,
myelofibrosis). Hemolytic anemias such as
thalassemia or sickle cell disease often lead to
cholelithiasis, requiring cholecystectomy at a
young age. The differential diagnosis for posterior
mediastinal masses includes neurogenic tumors,
lymphoma, hematoma, and abscess. Assembling all
the above findings leads to the diagnosis of
extramedullary hematopoiesis in the setting of
thalassemia. Extramedullary hematopoiesis occurs
in response to states of deficient bone marrow
productivity, such as anemia, myelofibrosis, and
leukemia. The reticuloendothelial system (spleen,
liver, and lymph nodes) is usually involved in
extramedullary hematopoiesis, but the posterior
mediastinum, skull, and kidneys can also be involved.
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Author:
D. Dean Thornton, MD, Clinical
Assistant Professor, Department of Radiology, University of Alabama at Birmingham
Advanced Imaging Associates of Alabama, Inc., HealthSouth Medical Center, Birmingham
eMedicine Editor:
Sat Sharma, MD
Associate Professor, University of Manitoba, Department of Medicine, Division of Pulmonary Medicine
Source
http://emedicine.com
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