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DIAGNOSIS PLEASE
Case 126: Extramedullary
Hematopoiesis1

䡲 CASE 126
Yahya M. Berkmen, MD†
History A 63-year-old man was admitted with a history of gradually
Benjamin A. Zalta, MD
increasing abdominal pain of 3 days duration. At admis-
sion, the pain was mostly localized to the right upper
quadrant. The patient had a slightly elevated white blood
cell count (10 ⫻ 109/L). The clinical impression of acute
cholecystitis was confirmed with ultrasonography and
computed tomography (CT). Several gallstones were also
depicted, and cholecystectomy was performed. Chest CT
studies were obtained postoperatively to evaluate an ab-
normality seen on the admission chest radiograph.

biliary tree on a lower section obtained

Imaging Findings
The posteroanterior chest radiograph
obtained at admission (Fig 1) showed a
smoothly outlined mass posterior to the
heart on the left side of the body. There
was also the hint of a retrocardiac opac-
ity on the right side. The heart was mod-
erately enlarged. Contrast material–en-
hanced CT of the chest was performed
after cholecystectomy and revealed
inhomogeneously enhancing bilateral
paraspinal masses in the lower half of
the thorax (Fig 2). Air was visible in the
Figure 1
Part one of this case appeared 4 months previously and may
contain larger images.
Published online
10.1148/radiol.2453040715
Radiology 2007; 245:905–908
1
From the Department of Radiology, Columbia University
Medical Center, 177 Fort Washington Ave, Milstein Hospi-
tal Bldg, MC 3, New York, NY 10032. Received April 22,
2004; revision requested June 29; revision received July
9; final version accepted July 28. Address correspon- Figure 1: Posteroanterior chest radiograph
dence to B.A.Z. (e-mail: baz2101@columbia.edu).
obtained at admission. There is a smoothly out-
Authors stated no financial relationship to disclose. lined mass (arrow) posterior to the heart on the left
†
side of the body. There is also suggestion of a
Deceased.
retrocardiac opacity on the right side of the body.
姝 RSNA, 2007
The heart is moderately enlarged.
through the midportion of the liver.
There were surgical clips in the left up-
per quadrant of the abdomen and the
spleen was absent, indicating previous
splenectomy.
Discussion
Extramedullary hematopoiesis (EMH) is
the proliferation of hematopoietic cells
outside of the bone marrow in response
to production of too few blood cells to
satisfy the body’s demand. This insuffi-
cient production is caused by either
bone marrow replacement disease (my-
elofibrosis or chronic myelogenous leu-
kemia) or hemolytic anemia (thalasse-
mia, sickle cell anemia, or hereditary
spherocytosis).
The findings of experimental studies
indicate that the organs develop from
specialized cells differentiated from one
totipotent cell but retain their ability to
dedifferentiate and proliferate into yet
another cell group. This explains how
neural cells can be generated from bone
marrow cells (1) and how muscle can be
regenerated by bone marrow– derived
myogenic progenitors (2). Conversely,
muscles and neural tissues can be in-
duced to produce hematopoietic cells
under certain experimental conditions
(3). It appears that “subpopulations of
cells within specific tissues may remain
unrestricted and possess pluripotent
potential that is reminiscent of embry-

Radiology: Volume 245: Number 3—December 2007 905

DIAGNOSIS PLEASE: Extramedullary Hematopoiesis
onic stem cells” (3). Extrusion of bone
marrow through a defect of severely
thinned and attenuated cortex of the
vertebrae or ribs in individuals with he-
molytic anemias may be another mech-
anism for development of EMH (4,5).
The liver, spleen, kidney, lymph
nodes, and posterior mediastinum are
the most common sites of EMH, with
EMH often occurring in more than one
site, although instances of isolated he-
patic (6) and renal (7) EMH have been
reported. EMH also may occur in un-
common locations, such as the skin (8),
central nervous system (9), adrenal
Figure 2
Figure 2: Transverse contrast-enhanced CT
images of the chest obtained after cholecystec-
tomy. (a) Inhomogeneously enhancing bilateral
paraspinal masses (M) at the lower end of the
thorax. (b) Lower section obtained through the
middle of the liver. Surgical clips in the left upper
quadrant (arrowhead) and absence of the spleen
are evident.
906
gland (10), middle ear (11), spine (12),
paratracheal region (13), abdomen
(14), and pelvis (15).
Intrathoracic EMH may manifest as
a paraosseous mass, interstitial pulmo-
nary abnormality, pleural mass, or he-
mothorax, either alone or in combina-
tion. Paraosseous EMH is the most com-
mon manifestation of intrathoracic EMH.
It appears as a small or large mass adja-
cent to the vertebrae (frequently bilater-
ally), ribs (commonly multiple ribs), or
both. These masses are usually asymp-
tomatic and may be discovered inciden-
tally (16,17). If specific clinical features
and radiologic evidence of myelofibrosis
or hemolytic anemia are present, this
disease is easy to diagnose. In the ab-
sence of specific bone changes associ-
ated with the underlying disease (my-
elofibrosis or anemia), the diagnosis can
be established with percutaneous fine-
needle aspiration (16,18), open biopsy
(14,19), or thoracoscopic biopsy (20)
in uncertain cases. EMH usually re-
gresses or disappears after treatment
with blood transfusion and hydroxyurea
(14), splenectomy in cases of spherocy-
tosis (21), or radiation therapy (13,19).
Chest radiography reveals a sharply
outlined, smooth, or lobulated (17)
paraspinal or rib mass(es). Paraspinal
EMH may be an isolated (16,17) or ex-
tensive mediastinal mass (5), or it may
be contiguous with abdominal paraspi-
nal masses of EMH (14). These lesions
homogeneously enhance on CT scans
obtained with intravenous contrast ma-
terial because of their high vascularity
(22). Iron deposition and fat infiltration
may occur in long-standing lesions (22),
and CT reveals an inhomogeneous con-
trast-enhanced mass, as in this case.
Inhomogeneity of the mass may be evi-
dent even without contrast enhacement
(21). Magnetic resonance imaging (22,23)
and technetium 99m sulfur colloid scan-
ning (18) are often useful diagnostic im-
aging modalities.
Inhomogeneously enhancing bilat-
eral paravertebral masses in patients
with myelofibrosis or hemolytic anemia
are highly indicative of EMH. These
masses have to be differentiated from
lymphadenopathy and neurogenic tu-
mors. Large posterior mediastinal lymph
Berkmen and Zalta
nodes can be found in patients with
Castleman disease (24). Typically, they
enhance homogeneously and intensely
and frequently have calcifications (24),
although inhomogeneous or ringlike en-
hancement has been reported in rare in-
stances (24,25). Calcification in patients
with EMH is demonstrated extremely
rarely (18,21). In tuberculosis, the lymph
nodes commonly enhance peripherally
(26); in lymphomas, enhancement is
usually faint and homogeneous. Neuro-
genic tumors of the posterior mediasti-
num commonly have erosions or defects
of the vertebrae, ribs, or both and may
show moderate homogeneous contrast
enhancement.
Involvement of the pulmonary inter-
stitium with hematopoietic cells causes
severe and often fatal respiratory failure
and rarely occurs (19,27). Chest radio-
graphic findings are described as “inter-
stitial lung infiltrates” (27). EMH masses
may form in the pleura and may be com-
plicated by hemothorax (28).
Hereditary spherocytosis is one of
several forms of congenital hemolytic
anemia. The erythrocytes acquire a spher-
ical shape because of a molecular defect
in one of the proteins of the red cell
membrane. Clinical findings are ane-
mia, jaundice, and splenomegaly. Gall-
stones occur frequently. Splenectomy is
the treatment of choice. With improve-
ment of the anemia, EMH regresses or
disappears. However, occasional per-
sistence of EMH after splenectomy, as
occurred in this patient, has been re-
ported (21). In conclusion, the combi-
nation of inhomogeneously enhancing
bilateral paraspinal masses at CT in a
patient with gallstones and previous
splenectomy makes EMH the most
likely diagnosis.
References
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3. Jay KE, Gallacher L, Bhatia M. Emergence of
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muscle and neural hematopoiesis in humans.
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Congratulations to the 150 individuals
and nine resident groups that submitted
the most likely diagnosis (extramedul-
lary hematopoiesis) for Diagnosis Please,
Case 126. The names and locations of the
individuals and resident groups, as sub-
mitted, are as follows:
Berkmen and Zalta
Individual responses
Jaleel P. Abdul, MD, Dammam, Saudi Arabia
Amr M. Ajlan, MBBS, Montreal, Quebec, Can-
ada
Oguz Akin, MD, New York, NY
Erhan Akpinar, Ankara, Turkey
Roger L. Antonelli, MD, Dayton, Ohio
Fahad Azzumeea, MBBS, Montreal, Quebec,
Canada
Asim K. Bag, MD, Birmingham, Ala
Ronald L. Becker, MD, Rolling Hills, Calif
Bryan D. Berkey, MD, Tripler AMC, Hawaii
J. Scott Bolton, MD, Dothan, Ala
Ronald N. Boyle, MD, La Plata, Md
Eric L. Bressler, MD, Minnetonka, Minn
Douglas C. Brown, MD, Virginia Beach, Va
Marcelo Cabrini, Lomas de Zamora, Argentina
Chris E. Chernesky, MD, Springfield, Mo
Michael H. Childress, MD, Silver Spring, Md
Alex T. Chuang, MD, Dallas, Tex
Ming-Tsung Chuang, MD, Kaohsiung, Taiwan
Neal R. Conti, MD, Seattle, Wash
Gonzalo L. Cortes, MD, Bilbao, Vizcaya, Spain
Marco A. Cura, MD, San Antonio, Tex
Paxton H. Daniel, MD, Fort Worth, Tex
Marc G. De Baets, MD, Collina d’Oro, Switzer-
land
Peter De Baets, Damme, Belgium
Johannes F. De Villiers, MBChB, MMed, Gis-
borne, New Zealand
Thaworn Dendumrongsup, MD, Songkla, Thai-
land
Bart D’herde, Hasselt, Belgium
Seyed A. Emamian, MD, PhD, Rockville, Md
Juliet H. Fallah, MD, Clarendon Hills, Ill
Susan M. Fanapour, DO, Lombard, Ill
Virginia Fattal Jaef, MD, Rosario, Argentina
Mario Fava, MD, Miami, Fla
Luis G. Ferrer, MD, Valencia, Spain
Angeles Franco Lopez, Madrid, Spain
Akira Fujikawa, MD, Setagaya, Tokyo, Japan
Ann S. Fulcher, MD, Midlothian, Va
Marcelo B. Funari, MD, São Paulo, Brazil
Rajneesh Galwa, Jaipur, India
Ram P. Galwa, MD, Chandigarh, India
Gilles Genin, Annecy, France
Carlos R. Gimenez, MD, New Orleans, La
Ferris Ginsberg, DO, Ocean, NJ
Francisco Jose Gonzalez, Santander, Spain
Bruno Miguel Graca, MD, Coimbra, Portugal
Aleksandar Grgic, MD, Zweibruecken, Germany
Dan G. Gridley, MD, Phoenix, Ariz
Flavius F. Guglielmo, MD, Basking Ridge, NJ
Pramod K. Gupta, MD, Plano, Tex
Adam R. Guttentag, MD, Philadelphia, Pa
Ehsan A. Haider, MBBCh, Montreal, Quebec,
Canada
Ferris M. Hall, MD, Brookline, Mass
Clare M. Hartigan, MBBCh, London, United
Kingdom
John J. Hines, Jr, MD, Huntington, NY
Hideki Hyodoh, MD, Sapporo, Hokkaido, Japan
Alberto C. Iaia, MD, Wilmington, Del
Teeranan Intharapat, MD, Hat-Yai, Songkhla,
Thailand
907
DIAGNOSIS PLEASE: Extramedullary Hematopoiesis
Rathachai Kaewlai, MD, Boston, Mass
Kiriakos Kalampoukas, MD, Halandri, Greece
Andrew J. Kapustin, MD, Charlotte, NC
Ashok Katti, MD, Warrington, Cheshire, United
Kingdom
Takuji Kiryu, MD, PhD, Gifu, Japan
Steven A. Klein, MD, Shrewsbury, Mass
Arlene M. Klink, MD, MS, Irvine, Calif
Stefanos Lachanis, Athens, Greece
Alexis Lacout, MD, Paris, France
James F. Lally, MD, Wallingford, Pa
Martin Lecompte, MD, Manotick, Ontario,
Canada
Karl J. Lehmann, MD, Karlsruhe, Germany
Myron M. Levitt, MD, Scotch Plains, NJ
Donald R. Lewis, Jr, MD, Huntington, WVa
John T. Lim, MD, Newport Coast, Calif
Eric D. Lindgren, MD, Grand Rapids, Mich
David A. Lisle, MBBS, Brisbane, Australia
Jaime Llauger, MD, Barcelona, Spain
Edward Lubat, MD, Englewood, NJ
Michael B. Martin, MD, Austin, Tex
Daniel L. Martins, MD, São Paulo, Brazil
Andrew C. Mason, MBBCh, Vancouver, British
Columbia, Canada
Satoshi Matsushima, MD, Tokyo, Japan
Waldir H. Maymone, MD, Rio de Janeiro, Brazil
Frank J. McKowne, MD, Vancouver, Wash
Sunil L. Mehta, MD, Mississauga, Ontario,
Canada
Jose M. Mellado Santos, Tudela, Navarra, Spain
Flavia Mendez, MD, Porto Alegre, Brazil
Michael P. Meyers, MD, Winnipeg, Manitoba,
Canada
Nikolaos Michailidis, Thessaloniki, Greece
Manabu Minami, MD, PhD, Yokohama, Japan
Mansour Mirfakhraee, MD, Shreveport, La
Tammam N. Nehme, MD, East Wenatchee,
Wash
Christopher C. Ng, MD, Nashville, Tenn
Soheil Niku, MD, San Diego, Calif
Mizuki Nishino, MD, Boston, Mass
908
Hiroshi Nobusawa, MD, PhD, Ota, Tokyo, Japan
Anietie E. Okon, MD, North Liberty, Iowa
Laura Oleaga, Philadelphia, Pa
Cinthia D. Ortega, MD, São Paulo, Brazil
Klaus Orth, Aachen, Germany
Marc G. Ossip, MD, Toronto, Ontario, Canada
Neeraj J. Panchal, MD, San Diego, Calif
David M. Panicek, MD, New York, NY
Matteo Passamonti, MD, Lodi, Italy
Narendrakumar P. Patel, MD, Newburgh, NY
Suresh K. Patel, MD, Chicago, Ill
Yeliz Pekcevik, Izmir, Turkey
Nicola Pelosi, Palmanova, Italy
Ivan Pilate, Brussels, Belgium
Rubem Pochaczevsky, MD, Bronx, NY
Ilias Primetis, MD, Athens, Greece
Daniel C. Rappaport, MD, Toronto, Ontario,
Canada
Matthew C. Rheinboldt, MD, Nashville, Tenn
Carla F. Ribeiro, MD, Viseu, Portugal
Walter S. Rose, MD, Oakhurst, NJ
Arnold J. Rotter, MD, Los Angeles, Calif
Tsutomu Sakamoto, MD, Tokyo, Japan
Roberto Q. Santos, MD, Rio de Janeiro, Brazil
Debra M. Sarasohn, MD, New York, NY
Anthony J. Scuderi, MD, Johnstown, Pa
Hidekazu Seo, MD, Hamamatsu, Shizuoka,
Japan
Matthew P. Shapiro, MD, Charlottesville, Va
Hideki Shima, MD, Tokyo, Japan
Grady V. Shue, Jr, MD, Hickory, NC
Ana C. Silva, MD, Porto, Portugal
Annamaria Skacelova, MD, Veazie, Me
Darrin S. Smith, MD, Visalia, Calif
Annemie Snoeckx, MD, Zandhoven, Belgium
Luis A. Sosa, Jr, MD, Mexico City, Mexico
James D. Sprinkle, Jr, MD, Spotsylvania, Va
Paul Stark, La Jolla, Calif
Evan Stein, MD, PhD, New York, NY
Subramanian Subramanian, MD, New Delhi, India
Norio Takahashi, MD, Fukui, Japan
Douglas L. Teich, MD, Brookline, Mass
Berkmen and Zalta
Osman Temizoz, Edirne, Turkey
Eugene Tong, MD, Austin, Tex
Vaiman S. Tsang, MD, Holtsville, NY
Baris I. Turkbey, MD, Baltimore, Md
Unni K. Udayasankar, MD, FRCR, Atlanta, Ga
Eleni Vafeiadou, Thessaloniki, Greece
Piet K. Vanhoenacker, MD, Moorsel, Belgium
Elida Vazquez, MD, Barcelona, Spain
Nanda Venkatanarasimha, MBBS, MRCP, Ply-
mouth, United Kingdom
Ricardo Luis Videla, Córdoba, Argentina
Christopher P. Vittore, MD, Belvidere, Ill
Ivan Vollmer, MD, Barcelona, Spain
Michael Weber, MD, Berlin, Germany
Jeffrey H. West, MD, Jacksonville, Fla
Satoru Yoshida, Muroran, Hokkaido, Japan
Kaneko You, Gifu, Japan
Stanko Yovichevich, MD, Sydney, Australia
Joe Yut, Olathe, Kan
Resident group responses
Baylor University Medical Center Radiology Res-
idents, Dallas, Tex
Fukushima University Radiology Residents, Fuku-
shima, Japan
Fundacion Jimenez Diaz Radiology Residents,
Madrid, Spain
Hospital Italiano Cordoba Radiology Residents,
Cordoba, Argentina
Hospital das Clinicas da Faculdade de Medicina
da Universidade de São Paulo Radiology
Residents, São Paulo, Brazil
Kaohsiung Medical University Radiology Resi-
dents, Kaohsiung, Taiwan
Prince of Songkla University Radiology Resi-
dents, Songkla, Thailand
University of Pennsylvania Radiology Residents,
Philadelphia, Pa
Virginia Commonwealth University Radiology
Residents, Richmond, Va
Radiology: Volume 245: Number 3—December 2007