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Typical and Atypical Presentations of Extramedullary Hemopoiesis

Christos S. Georgiades1, Edward G. Neyman1, Isaac R. Francis2, Michael B. Sneider2 and Elliot K. Fishman1

1 Russell H. Morgan Department of Radiology and Radiological Sciences, Johns Hopkins University School of Medicine and Johns Hopkins Hospital, 601 N. Caroline St., Baltimore, MD, 21287.
2 Department of Radiology, University of Michigan Medical School, 1301 Catherine Rd., Ann Arbor, MI 48109.



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  		Fig. 1A. —Graphs show sites of normal hemopoiesis. In embryo and fetus, hemopoiesis is initially localized in yolk sac. Within 1-2 months, liver and spleen begin physiologic extramedullary hemopoiesis, followed by bone marrow. By birth all extramedullary hemopoiesis ceases.

 


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  		Fig. 1B. —Graphs show sites of normal hemopoiesis. After birth, all normal hemopoiesis occurs in bone marrow of proximal long bones, ribs, sternum, and vertebrae [1], which fail over time in that same order.

 


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  		Fig. 2A. —23-year-old woman with history of thalassemia and known extramedullary hemopoiesis. Axial contrast-enhanced CT scan through chest shows uniformly enhancing paraspinal hemopoietic masses with no bony erosion.

 


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  		Fig. 2B. —23-year-old woman with history of thalassemia and known extramedullary hemopoiesis. Posteroanterior chest radiograph shows well-marginated bilateral, paraspinal masses compatible with extramedullary hemopoietic tissue.

 


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  		Fig. 2C. —23-year-old woman with history of thalassemia and known extramedullary hemopoiesis. Lateral chest radiograph shows paraspinal location of these masses.

 


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  		Fig. 3A. —25-year-old man with ß-thalassemia. Posteroanterior chest radiograph shows diffuse expansion of ribs and right upper paraspinal thoracic mass (arrow) compatible with extramedullary hemopoiesis.

 


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  		Fig. 3B. —25-year-old man with ß-thalassemia. Lateral skull radiograph shows characteristic expansion and hairon-end appearance of diploic space.

 


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  		Fig. 4A. —54-year-old woman with long-standing history of myelofibrosis who presented with abdominal discomfort and gradual onset of shortness of breath. Posterior (A) and anterior (B) views from 99mTc methylene diphosphonate wholebody bone scans show diffusely increased activity in massively enlarged liver that compromises lung volume.

 


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  		Fig. 4B. —54-year-old woman with long-standing history of myelofibrosis who presented with abdominal discomfort and gradual onset of shortness of breath. Posterior (A) and anterior (B) views from 99mTc methylene diphosphonate wholebody bone scans show diffusely increased activity in massively enlarged liver that compromises lung volume.

 


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  		Fig. 5. —51-year-old woman with myelofibrosis. Abdominal discomfort prompted clinic visit during which physical examination revealed large spleen. Coronal T1-weighted MR image shows massively enlarged spleen. Patient's symptoms worsened over time, and splenic biopsy was followed by splenectomy. Pathologic examination revealed diffuse infiltration with immature and mature heme elements, which is compatible with extramedullary hemopoiesis.

 


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  		Fig. 6A. —40-year-old man with sickle cell disease and frequently recurring painful crises who presented with abdominal pain. Axial unenhanced CT scan at thoracoabdominal level reveals two uniformly low-attenuation (compared with liver parenchyma), well-marginated lesions (arrows). Percutaneous biopsy was diagnostic for foci of extramedullary hemopoiesis.

 


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  		Fig. 6B. —40-year-old man with sickle cell disease and frequently recurring painful crises who presented with abdominal pain. Axial unenhanced CT scan more caudal than A shows diffusely increased density of liver parenchyma. Differential diagnosis included primary or secondary hemochromatosis or hemosiderosis, treatment with amiodarone, and Wilson's disease.

 


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  		Fig. 7A. —53-year-old man with weight loss and abdominal pain. Axial T1-weighted MR image shows hypointense exophytic mass (arrow) from left lobe of liver.

 


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  		Fig. 7B. —53-year-old man with weight loss and abdominal pain. Axial T2-weighted MR image at same level as A shows mass to be slightly hyperintense to liver parenchyma. Percutaneous biopsy showed extramedullary hemopoiesis. Patient was subsequently diagnosed with myelofibrosis.

 


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  		Fig. 8A. —56-year-old man with myelofibrosis who presented with flank and abdominal pain. Axial unenhanced CT scan through upper abdomen obtained 2 years previously shows no abnormality.

 


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  		Fig. 8B. —56-year-old man with myelofibrosis who presented with flank and abdominal pain. Axial contrast-enhanced CT scan through kidneys from current admission reveals bilaterally symmetric enhancing perinephric masses. Biopsy showed extramedullary hemopoiesis.

 


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  		Fig. 9A. —33-year-old man with unspecified myeloproliferative disorder who presented with abdominal pain. Patient has history of splenectomy for severe splenomegaly and cavernous transformation of portal vein. Axial contrast-enhanced CT scan through upper abdomen shows ill-defined mass (arrow) in porta hepatis infiltrating mesentery along gastrohepatic ligament.

 


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  		Fig. 9B. —33-year-old man with unspecified myeloproliferative disorder who presented with abdominal pain. Patient has history of splenectomy for severe splenomegaly and cavernous transformation of portal vein. Axial gadolinium-enhanced T1-weighted fat-suppressed MR image at same level as A shows same findings without adding specificity. Percutaneous biopsy revealed immature hemopoietic elements compatible with extramedullary hemopoiesis.

 


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  		Fig. 10. —48-year-old man with history of hemolytic anemia and myelofibrosis. Axial unenhanced CT scan through pelvis shows well-marginated presacral soft-tissue mass (arrow) with no bony erosion. Biopsy was diagnostic of extramedullary hemopoiesis.

 

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