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7.9 Sjögren Syndrome (Myoepithelial Sialadenitis) 283
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Signs on CT
In the acute phase, the aff ected glands are bilaterally enlarged
with parenchymal nodules of variable size and formation of
cysts, giving a honeycomb appearance (Fig. 7.9.2 ).
The salivary glands often show signs of fi brosis and size
shrinkage in advanced stages of the disease.
The lacrimal glands might be bilaterally and symmetrically
enlarged (Fig. 7.9.3 ).
Fig. 7.9.3. Sequential nonenhanced orbital CT images of the
same patient with Sjögren syndrome (SS) show bilateral lacrimal glands enlargement ( arrows )
Signs on Brain MRI
Brain MRI shows multifocal T2 hyperintensities located in the
subcortical and periventricular white matter (Fig. 7.9.4 ),
enlargement of the sulci, and ventricular dilatation. These
manifestations are often seen in 50% of patients with focal
neurological defi cits or psychiatric manifestations.
Fig. 7.9.2. Sequential postcontrast head CT images ( a ) and ( b )
of a patient with Sjögren syndrome (SS) show bilateral parotid
gland bulging and enlargement ( arrows )
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7.9
Fig. 7.9.5. Axial T1W ( a ) and T2W ( b ) images of a patient with
Sjögren syndrome (SS) show bilateral moderate parotid glands
enlargement with fi ne, small cysts formation within the gland
( arrowheads )
Fig. 7.9.4. Axial FLAIR brain MR illustration demonstrates
patchy areas of high T2 signal intensity lesions within the white
matter as presentation of neuro-Sjögren syndrome (SS)
Signs on Parotid MRI
Bilateral, almost symmetrical enlargement of the parotid
glands (Fig. 7.9.5 ).
Low T1/high T2 multiple cysts are seen within the parotid and
lack of enhancement (“salt and pepper” appearance) (Fig. 7.9.5 ).
Chronic disease can lead to gland shrinkage, microcysts
formation, and calcifi cations.
For Further Reading
1. Mataró M et al Magnetic resonance abnormalities associ-
ated with cognitive dysfunction in primary Sjögren syndrome. J Neurol. 2003;250:1070–6
2. Mizuno Y et al Recurrent parotid gland enlargement as an
initial manifestation of Sjögren syndrome in children. Eur
J Pediatr. 1989;148:414–16
3. Kobayashi I et al Complications of childhood Sjögren syndrome. Eur J Pediatr. 1996;155:890–4
4. Tristano AG et al A case of Sjögren’s syndrome with acute
transverse myelitis and polyneuropathy in a patient free of
sicca symptoms. Clin Rheumatol. 2005;25:113–14
5 . A d ž i ć TN et al Multinodular pulmonary amyloidosis in pri-
mary Sjögren’s syndrome. Eur J Int Med. 2008;19:e97–8
6. Ohbayashi N et al Sjögren syndrome: Comparison of
assessments with MR sialography and conventional sialography. Radiology 1998;209:683–8
7. Yoon YH et al Sialectasis of Stensen’s duct: an unusual case
of recurrent check swelling. Eur Arch Otorhinolaryngol.
doi: 10.1007/s00405–008–0702–0
8. Varghese JC et al A prospective comparative study of MR
Sialography and conventional sialography of salivary duct
disease. AJR 1999;173:1497–503
9. Kalk WWI et al Parotid sialography for diagnosing Sjögren
syndrome. Oral Surg Oral Med Oral Pathol Oral Radiol
Endod. 2002;94:131–7
10. Kassan SS et al Clinical manifestations and early diagnosis
of Sjögren’s syndrome. Arch Intern Med. 2004;164:1275–84
11. Madani G et al Infl ammatory conditions of the salivary
glands. Semin Ultrasound CT MRI. 2006;27:440–51
7.10 Behçet Disease 285
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7.10
Behçet Disease
Behçet disease (BD) is a relatively rare rheumatological disease characterized by the triad of apthus oral
ulcers, genital ulcers, and ocular infl ammation (uveitis).
The disease is named after its fi rst describer, the
Turkish dermatologist Hulusi Behçet, in 1937.
Clinical Criteria to Diagnose BD Require
a Combination of Three or More
of the Following Findings
Recurrent aphthous stomatitis (79%). The lesions
are punched out with rolled edges.
Recurrent genital ulcers, mainly seen on the scrotum
or labial majora, which heal by scar formation.
Anterior or posterior uveitis, presenting with pain,
blurry vision, and redness.
Vasculitis of cutaneous or large vessels.
Mono-or oligoarthritis affecting the knees in
particular.
Meningoencephalitis.
Cutaneous hyperactivity to minor trauma.
BD lesions are characterized by chronic infl ammation
of the soft tissues with neutrophilic infi ltration, which
is characteristic of BD lesions regardless of the disease
stage.
BD is a multisystemic disease with many manifestations. Documented complications of BD are those
that affect the gastrointestinal tract (GI), large vessels
(vasculitis and thrombophlebitis), muskuloskeletal
system (myonecrosis and arthritis), renal system (proteinuria and hematuria), and the nervous system
(neuro-BD).
Myonecrosis should be considered in patients with
known BD presenting with acute onset of muscle pain
and edema in the absence of signs and symptoms of
infection. Renal BD is mainly caused by secondary
amyloidosis (AA-type) and glomerulonephritis.
Neuro-BD has three patterns of presentation: the
fi rst pattern is seen as brain parenchymal lesions presenting in stroke-like lesions and brain stem syndrome;
the second pattern is migraine headache (64%), papilledema, and increased intracranial hypertension; and the
third pattern presents in the form of meningitis-like
disease.
The esophagus is involved in 50% of patients usually in its mid-portion. Patients with esophageal
involvement often present with substernal pain, dysphagia, and occasional hematemesis. Esophageal
varices may develop when the superior vena cava is
obstructed due to thrombophlebitis ( superior vena
cava syndrome ). Thrombophlebitis may involve the
hepatic veins resulting in Budd–Chiari syndrome (liver
congestion and cirrhosis due to hepatic veins outfl ow
obstruction).
GI manifestations of BD may mimic the manifestations of Crohn’s disease (CD) radiologicaly, and even
pathologically. However, involvement of the rectum
and anus is rare in BD; perforation is more common in
BD than CD, no cobbelstoning in BD, and the GI disease is usually milder in BD than in CD.
Diagnosis is essentially clinical, and atypical presentation can be misleading and delay the diagnosis.
Signs on Enteroclysis
Distal ileum infl ammation and aphthous ulceration are seen in
up to 80% of patients with GI manifestations of BD.
Pseudopolyp formation is seen in 35% of cases.
Signs on MRI
In the muscles, myonecrosis presents with soft tissue
infl ammation in the form of soft tissue mass with high signal
intensity on T2W images and rim contrast enhancement on
postgadolinium injection images.
In the brain, multiple, round, small (>5 mm) white matter
high T2 signal intensity lesions on FLAIR and T2W images,
usually in the juxtacortical region (Fig. 7.10.1 ). Brain stem
lesions and atrophy can be seen.
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7.10
Fig. 7.10.1. Axial FLAIR brain MR illustration demonstrates
the neurological fi ndings in neuro-Behçet disease
For Further Reading
1. Stubbs AY et al Myonecrosis in Behcet’s disease. Skeletal
Radiol. 2008;37:357–60
2. Hwang I et al Necrotizing villitis and decidual vasculitis in
placentas of mothers with Behçet disease. Human Pathol.
2009;40:135–8
3. Benjilali L et al Chylothorax and chylopericardium in a
young man with Behçet disease. Joint Bone Spine 2008;75:
740–52
4. Akpolat T et al Renal Behçet’s disease: an update. Semin
Arthritis Rheum. 2008;38:241–8
5. Korman U et al Enteroclysis fi ndings of intestinal Behcet
disease: a comparative study with crohn disease. Abdom
Imaging 2003;28:308–12
6. Oktay Kaçmaz Ret al Ocular infl ammation in Behçet dis-
ease: incidence of ocular complications and loss of visual
acuity. Am J Opthalmol. 2008;146:828–36
7. Ebert EC et al Gastrointestinal manifestations of Behçet’s
disease. Dig Dis Sci. doi: 10.1007/s10620–008–0337–4
8. Bank I et al Dural sinus thrombosis in Behçet’s disease.
Arthritis Rheum. 1984;27:816–8
9. La Mantia L et al Headache and infl ammatory disorders of
the central nervous system. Neurol Sci. 2004;25:S148–53
10. Jäger HR et al MRI in neuro-Behcet’s syndrome:
Comparison of conventional spin-echo and FLAIR pulse
sequences. Neuroradiology 1990;41:750–8
7.11 Sharp Syndrome (Mixed Connective Tissue Disease) 287
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7.11
Sharp Syndrome (Mixed Connective
Tissue Disease)
Mixed connective tissue disorder (MCTD) is a rare
disease characterized by a combination of clinical features similar to systemic lupus erythematosus (SLE),
scleroderma, and polymyositis, and unusually high
titers of antibody to RNase-sensitive ribonuclear protein complex (RNP).
MCTD patients have mixed features of rheumatological symptoms that do not match a certain rheumatological category. For example, polyarthritis, myositis,
and hypergammaglobulinemia are more common in
MCTD than in scleroderma. Also, polyarthritis and
hypergammaglobulinemia are more frequently found
in MCTD than in polymyositis. Esophageal hypermotility, pulmonary hypertension, and absence of renal
disease are typical features of MCTD. Moreover, the
detection of serological antibodies to RNP is very specifi c for MCTD, and very rarely found in other rheumatic or connective tissue disorders.
Patients with MCTD may show neurological signs
like seizures, psychosis, headache, gait disturbance,
and polyneuritis. Trigeminal neuralgia has been
reported in patients with MCTD, and seen frequently
when the patient has more symptoms toward scleroderma. In contrast, MCTD with more symptoms toward
SLE shows neurological symptoms like gait ataxia,
transverse myelitis, and optic neuropathy. MCTD is
one of the rare causes of “treatable dementia.”
MCTD should be considered in a patient when his/
her symptoms and signs cannot be confi ned to a sole
rheumatological disease.
Signs on Radiograph and CT
Chest radiograph or CT may show signs of pulmonary hypertension (e.g., prominent pulmonary trunk) (Fig. 7.11.1 ).
Signs on Brain MRI
Optic or trigeminal neuritis is seen as contrast enhancement
of the nerve after gadolinium injection due to the hyperemia
and infl ammation (the normal nerve does not enhance after
contrast injection).
Transverse myelitis shows enlargement of the cord with
diff use hyperintense signal of the spinal cord on T2W images
in the aff ected segment (Fig. 7.11.2 ). The aff ected segment
enhances in an inhomogeneous pattern after contrast
administration. Cord atrophy occurs in chronic cases. On axial
segments, the high signal on T2W images occurs on both
sides of the cord giving a “snake-eye appearance.”
Fig. 7.11.1. Axial postcontrast CT-angiography of the pulmonary vessels in 32-year-old woman with Sharp’s syndrome
shows mildly dilated pulmonary trunk (28 mm) due to newly
developed pulmonary hypertension ( arrowhead )
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7.11
Fig. 7.11.2. Sagittal T1W postcontrast cervical spine MRillustration demonstrates transverse myelitis as thickened spinal
cord with
For Further R eading
1. Colombo A et al Mixed connective tissue disease (Sharp
syndrome): Description of two cases. Ital J Neurol Sci.
1983;2:203–5
2. Matsui H et al Encephalopathy and sever neuropathy due to
probable systemic vasculitis as an initial manifestation of
mixed connective tissue disease. Neurol India. 2006;54:
83–5
3. Malaviya AN et al Sharp’s syndrome (mixed connective tissue disease) with extensive infl ammatory panniculitis
complicated with pyoderma gangrenosum–a case report.
J Indian Rheumatol Assoc. 2003;11:45–50
Chapter 8
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Hematology
CONTENTS
8.1 Hemosiderosis and Hemochromatosis 290
8.2 b-Thalassemia Major (Cooley’s Anemia) 293
8.3 Sickle Cell Disease 297
8.4 Pernicious Anemia 301
8.5 Hemophilia 303
8.6 Lymphomas 306
8.7 Leukemia 313
8.8 Multiple Myeloma (Khaler’s Disease) 319
8.9 Amyloidosis 323
8.10 Evans’ Syndrome 328
8.11 Other Lymphatic Disorders 329
J. A. Al-Tubaikh: Internal Medicine – An Illustrated Radiological Guide
DOI: 10.1007/978-3-642-03709-2_8, © Springer-Verlag Berlin Heidelberg 2010
289
290 Chapter 8 Hematology
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8.1
8.1
Hemosiderosis and Hemochromatosis
Hemosiderosis , or iron overload, is a pathological con-
dition characterized by deposition of excess iron within
the body tissues that normally do not containing iron.
Hemosiderosis is usually secondary to a primary cause
such as multiple blood transfusion, chronic hemodialysis, or hemolytic anemia (e.g., thalassemia).
When iron is released into the cytoplasm, it enters a
cellular compartment called “labile iron pool” (LIP).
Both ferrous (Fe
bound with proteins, and are highly toxic to the cells
within this compartment. The unneeded iron from LIP is
stored in the form of ferritin, which is organic and nontoxic. When the LIP iron content exceeds the ferritin
capacity, hemosiderin is generated from ferritin denaturation. Iron in the form of hemosiderin is thought to be
more toxic to the body tissues. Hemosiderin initially
accumulates in the reticuloendothelial system (spleen,
bone marrow, and Kupffer cells in the liver). When the
reticuloendothelial system is saturated, deposition occurs
in normal body tissues such as the hepatocytes, heart
muscles, and the endocrine system. Chelation therapy
(e.g., desferrioxamine) removes mainly the extracellular
iron and only a fraction of the intracellular LIP iron.
Hemosideroses have different body manifestations
according to the area of deposition:
Cardiac hemosiderosis : myocardial iron deposition
results in dilated cardiomyopathy that will lead to
heart failure.
2+
) and ferric (Fe 3+ ) iron forms are poorly
Fig. 8.1.1. Short-axis cardiac MRI illustration demonstrates a
black ring within the myocardium, a sign of cardiac siderosis
( arrowhead )
Hepatic hemosiderosis : iron deposition in hepato-
cytes results in liver cirrhosis.
Signs on CT and MRI
On CT, the liver appears hyperdense compared to the spleen.
On MRI, the liver appears extremely hypointense, with an
almost black signal on all pulse sequences, depending on the
severity of the iron overload.
Anterior pituitary gland hemosiderosis : loss of the endocrine
function of the anterior pituitary results in hypogonadism and
loss of libido.
Signs on Chest Radiograph
The heart appears larger than normal in cases of cardiac damage
and development of dilated cardiomyopathy.
Signs on MRI
MRI can detect early myocardial siderosis via obtaining T2*
images, which show a dark, hypointense rim located within the
myocardium in short-axis sequences, representing myocardial
siderosis (Fig. 8.1.1 ).
Signs on MRI
The anterior pituitary shows a hypointense signal intensity area
on both T1 and T2 images.
Pancreatic hemosiderosis : deposition of iron in the
pancreases results in impairment of both exocrine
and endocrine functions. Diabetes mellitus may
result due to pancreatic siderosis.
8.1 Hemosiderosis and Hemochromatosis 291
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Juvenile hemochromatosis is a form of hemochro-
Signs on US
The normal pancreas has almost the same echogenicity as the
liver on ultrasound. In siderosis, the pancreas may appear
hyperechoic compared to the liver, due to iron overload. Other
causes of hyperechoic pancreas include fatty infi ltration and
pancreatic calcifi cation.
Signs on MRI
Hypointense signal intensity areas are seen within the pancreas
on both T1W and T2W images ).
Hemochromatosis is a disease characterized by depo-
sition of excess iron in the body as a result of genetic
defect (primary hemosiderosis).
In hemochromatosis, iron deposition initially occurs
in the hepatocytes and spares Kupffer cells (the reverse
situation to hemosiderosis). The age of presentation is
usually between 50 and 60 years of age. Menstruation
blood loss in women has a protective effect against
hemochromatosis due to iron loss.
Hemochromatosis is asymptomatic in early stages.
As the iron deposition progresses, liver failure, skin
hyperpigmentation, arthropathy (especially in the metacarpophalangeal joints), and cardiac failure may occur.
Deposition of hemosiderin in the subcutaneous tissues
results in increased skin tanning and dark ening.
Bronze diabetes is a term used to describe maturity-
onset diabetes seen in hemochromatosis. The term
“bronze” is used because the diabetes is associated
with skin tanning that mimics bronze coloring.
Diagnosis is confi rmed by measuring serum ferritin
level, transferrin saturation testing, liver biopsy, genetic
testing, and MRI.
D i ff erential Diagnoses and Related Diseases
Bantu siderosis a type of hemosiderosis only found
in Africa. It is associated with liver cirrhosis, diabetes, and heart disease. The disease is linked with
higher rates of tuberculosis infection.
matosis that present in the second decade of life.
Patients often present with abdominal pain, cardiac
arrhythmias, impaired glucose tolerance, and hypogonadotrophic hypogonadism.
Ferroportin disease is a genetic disease character-
ized by mutation in the gene responsible for the production of ferroportin, a protein that exports iron
from body cells. Ferroportin is expressed mainly in
Kupffer cells and splenic macrophages. Patients
present with isolated hyperferritinaemia and normal
or slightly elevated transferrin saturation. In contrast
to hemochromatosis, iron is mainly deposited within
Kupffer cells.
Pulmonary hemosiderosis is a rare condition that
arises due to repeated episodes of bleeding within
the lung alveoli. Iron overload within the lungs
results in pulmonary fi brosis, anemia, and (rarely)
death due to pulmonary hemorrhage. The disease
has an incidence of less than 1:1,000,000 live births,
and occurs usually in children <7 years old (it is
extremely rare in adults). Symptoms include coughing blood (hemoptysis) and iron-defi ciency anemia.
Superfi cial brain siderosis : superfi cial siderosis of
the brain is a condition characterized by deposition
of hemosiderine within brain tissues, often secondary to subdural hemorrhage and bleeding into the
brain cisterns. When siderosis affects the vestibulocochlear nerve, tinnitus may result.
Signs on MRI
The liver appears extremely hypointense on all pulse
sequences, depending on the iron overload severity.
Muscles and kidneys may appear hyperintense, due to iron
deposition.
The spleen is characteristically spared and unaff ected in
hemochromatosis. In contrast, the spleen is almost always
aff ected in hemosiderosis.
In superfi cial brain siderosis , a characteristic band of low T1
and T2 signal intensity is seen around the brain tissue,
especially the pons, due to its location within the pontine
cistern (Fig. 8.1.2 ).
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Fig. 8.1.2. Axial T1W ( a )
and T2W ( b ) brain MRI
show a hypointense rim
that surrounds the pons
( arrowhead ) due to superfi -
cial brain siderosis
8.1
For Further Reading
1. Rosenberg W. Haemochromatosis. Medicine. 2007;35:89–92
2. Rosenberg W et al Haemochromatosis. Medicine. 2002;30:
63–4
3. Koçak R et al The liver siderosis in beta-thalassemia inter-
media and hemoglobin disease. J Islamic Acad Sci. 1993;6:
42–5
4. Flyer MA et al Transfusional hemosiderosis in sickle cell
anemia: another cause of an echogenic pancreas. Pediatr
Radiol. 1993;23:140–42
5. Brasch RC et al Magnetic resonance imaging of transfu-
sional hemosiderosis complicating thalassemia major.
Radiology. 1984;150:767–71
6. Chen CH et al Idiopathic pulmonary hemosidernossis:
favorable response to corticosteroid. J Chin Med Assoc.
2008;71:421–24
7. Deugnier Y et al Iron and the liver: update 2008. J Hepatol.
2008;48:S113–23
8. Argyropoulou MI et al MRI evaluation of tissue iron burden in patients with b -thalassemia major. Pediatr Radiol.
2007;37:1191–200
9. Bonetti MG et al Hepatic iron overload in thalassemic
patients: proposal and validation of an MRI method of
assessment. Pediatr Radiol. 1996;26:650–56
10. Positano V et al Improved T2* assessment in liver iron
overload by magnetic resonance imaging. Magn Reson
Imaging. 2008. doi:10.1016/j.mri.2008.06.004
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