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Chapter 9 · Hematology
5 CNS chloroma is seen as an intermediate to
hyperdense lesion (60–80 HU), with typically
homogenous enhancement after contrast injection
(nonspecific pattern). Mild to moderate
hypervascularity can be seen on CT angiography.
5 Small parenchymal brain calcifications can be seen
after episodes of intracranial radiation therapy.
9
. Fig. 9.7.30 Coronal postcontrast neck CT of a patient with
neck chloroma shows bilateral lymphadenopathy ( arrowheads )
Signs on MRI
5 Diffuse vertebral bone marrow leukemic
infiltration results in low signal intensity of the
vertebral bodies in relation to the intervertebral
disks ( bright disk sign ) (. Fig. 9.7.31 ).
Hematopoietically active marrow is referred to as
“red marrow.” Red marrow is composed of water
(40 %), fat (40 %), and proteins (20 %). In contrast,
hematopoietically inactive marrow is referred to as
“yellow marrow.” Yellow marrow is composed of fat
(80 %), water (15 %), and proteins (5 %). The normal
yellow marrow is hyperintense on T1W images.
Normal red marrow is generally hypointense on
MRI compared to yellow marrow, but its signal is
generally greater than that of muscle. It is perhaps
difficult to distinguish red marrow from infiltrative
marrow processes. Infiltrative bone marrow
pathology is any pathology that replaces the
normal bone marrow contents. This pathological
process can be diffuse or focal; neoplastic disease
(e.g., leukemia) represents the most common
etiology for vertebral bone marrow infiltration.
5 CNS chloroma shows low T1 and high T2 signal
intensities with marked homogenous
enhancement after contrast injection
(. Fig. 9.7.32 ).
5 Methotrexate leukoencephalopathy is seen as areas
of high signal intensities with no contrast
enhancement after contrast injection
(. Fig. 9.7.33 ).
5 I n disseminated necrotizing leukoencephalopathy ,
there are multiple areas of high T2 signal
intensities with small irregular low T2 signal foci
due to coagulative necrosis. The small low T2
signal foci show enhancement after contrast
injection.
5 Superior sagittal sinus thrombosis is seen as a
triangular filling defect on sagittal images ( empty
delta sign ). Sinus thrombosis can also be seen on
T2W images as a hyperintense vessel
(. Fig. 9.7.34 ). The vessel loses its void signal and
appears clearly due to the thrombosed
intravascular blood. The thrombus can show
contrast enhancement if it is old and organized.
9.7 · Leukemia
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9
a
b
. Fig. 9.7.31 Sagittal T1W thoracolumbar MRI in a normal patient ( a ) and a patient with leukemia, with di use vertebral bone marrow
in ltration, shows the classic bright disk sign in ( b ). Notice how the vertebral body shows higher signal intensity than the intervertebral
disk in ( a ) due to the presence of yellow marrow. In ( b ), the vertebral body shows lower signal intensity than the intervertebral disk, which
is described as the bright disk sign
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Chapter 9 · Hematology
a
b
9
. Fig. 9.7.32 Axial native T1W ( a ) and T1W postcontrast ( b ) brain MRI of a patient with biopsy-proved chloroma shows a large occipital
mass with a relatively hypointense signal on the left of the T1W image, with homogenous marked enhancement ( arrowheads )
a
. Fig. 9.7.33 Axial T1W ( a ), T2W ( b ), and T1W postcontrast brain MRI of a patient with ALL treated with methotrexate show di use
leukoencephalopathy of the centrum semi-ovale bilaterally ( b ), with no contrast enhancement in ( c )
b
c
9.7 · Leukemia
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a
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b
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. Fig. 9.7.34 Axial T1W postcontrast ( a ) and T2W ( b ) brain MRI of a patient with ALL treated with chemotherapy, who developed right
transverse sinus thrombosis, show lling defect in ( a ) ( arrowhead ), and hyperintense vessel in ( b ) due to the intravascular thrombus
( arrow )
Further Reading
Alkubaidan FO, etal. Granulocytic sarcoma (chloroma) of
the shoulder in Shwachman-Diamond syndrome. Eur J
Radiol Extra. 2007;64:107–10.
Carroll KW, etal. Useful internal standards for distinguish-
ing in ltrative marrow pathology from hematopoietic
marrow at MRI.J Magn Reson Imaging. 1997;7:394–8.
Cretzula JC, etal. Bloom’s syndrome. J Am Acad Dermatol.
1987;17:479–88.
Enright H, et al. Chronic leukemias. Dis Mon. 2008;54:
242–55.
Faber J, etal. Shwachman-diamond syndrome: early bone
marrow transplantation in a high risk patient and new
clues to pathogenesis. Eur J Pediatr. 1999;158:995–1000.
Frohna BJ, et al. Granulocytic sarcoma (chloroma) causing
spinal cord compression. Neuroradiology. 1993;35:509–11.
Hermann G, etal. Skeletal manifestations of granulocytic
sarcoma (chloroma). Skeletal Radiol. 1991;20:509–12.
Jacobs P. Myelodysplasia and leukemias. Dis Mon.
1997;43:505.
Jain D, etal. Bloom syndrome in sibs: rst report of hepato-
cellular carcinoma and Wilms tumor with documented
anaplasia and nephrogenic rests. Pediatr Dev Pathol.
2001;4:585–9.
Kolitz JE. Acute leukemia in adults. Dis Mon. 2008;54:
226–41.
Laningham FH, etal. Childhood central nervous system leu-
kemia: historical perspectives, current therapy, and acute
neurological sequelae. Neuroradiology. 2007;49:
873–88.
Lee YH, etal. Granulocytic sarcoma (chloroma) presenting
as a lateral neck mass: initial manifestation of leukemia: a
case report. Eur Arch Otorhinolaryngol. 2006;263:16–8.
O’Brien MM, etal. Acute leukemia in children. Dis Mon.
2008a;54:202–25.
O’Brien J, etal. An unusual cause of persistent headache:
chloroma (2008:2b). Eur Radiol. 2008b;18:1071–2.
Pande AR, etal. Disseminated necrotizing leukoencepha-
lopathy following chemoradiation therapy for acute lymphoblastic leukemia. Radiat Med. 2006;24:515–9.
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Chapter 9 · Hematology
9.8 Multiple Myeloma (Kahler’s Disease)
the renal function, as it can result in renal failure and death in
some MM patients.
Multiple myeloma (MM) is a malignant disease characterized by neoplastic proliferation of plasma cell precursors in
the bone marrow. e disease can arise di usely or focally in
any region in the body. e focal form of multiple myeloma
is called plasmacytoma . MM is a disease of older age groups
and typically found in patients between 40 and 70 years of
age.
e cardinal features of MM are osteolytic lesions found
on plain X-rays, anemia, proteinuria, and bone pain. Other
features include weight loss, anorexia, hepatosplenomegaly
(25 %), high serum alkaline phosphatase level, and high
erythrocyte sedimentation rate (ESR). Peripheral blood
smears show characteristic stacking of the red blood cells
(Rouleaux formation).
Anorexia in MM has been attributed to the toxic e ects
from breakdown products, and pain is o en intense, requiring narcotics. Pain in MM can arise due to di erent mecha-
9
nisms such as bone pain due to expansion of the bone
marrow by the myelomatous tissue, root pain caused by
compression or direct invasion of the nerve roots, periarticular pain mimicking arthritis, and pathologic bone fractures. Back pain commonly arises due to vertebral
pathologic fractures and collapse. Back pain in MM is made
worse by turning or twisting and is aggravated by coughing
or sneezing.
In MM, signs of amyloidosis may be seen in the form of
macroglossia, skin papules, and alopecia. Raynaud’s phenomenon, cold urticaria, and necrosis of the skin may be seen due
to cryoglobulinemia. Cryoglobulinemia is a condition charac-
terized by the presence of large amounts of proteins that
become insoluble at reduced temperature (e.g., 4 °C).
Increased plasma osmolarity due to the high plasma cell content in the blood may cause impairment of cerebral circulation due to increased plasma viscosity, a rare condition
known as paraproteinemic coma .
One of the most dramatic complications of MM is the
sudden compression of the spinal cord by collapsed vertebra
or plasmacytoma. Vertebral plasmacytoma arises from the
bone marrow and tunnels through the cortex until it spreads
Rarely, multiple myeloma of the mandible may present
with paresthesia of the chin and the lower lip due to in ltration of the mental nerve, a branch of the third division of
trigeminal nerve, when the mandible is a ected by multiple
myeloma. e condition is known as numb chin syndrome ,
and it is seen in malignancy that involves the mandible (e.g.,
leukemia).
POEMS syndrome , also known as Crow – Fukase syndrome ,
is a rare plasma cell disease with multisystemic involvement
characterized by p olyneuropathy, o rganomegaly, e ndocri-
nopathy, m onoclonal gammopathy, and s kin changes.
Patients with POEMS syndrome initially present with typical
symptoms of connective tissue disorder, such as sclerodermalike skin thickening. Other manifestations include hepatosplenomegaly, Castleman’s disease lymphadenopathy,
hypothyroidism, hypogonadism, peripheral sensory-motor
polyneuropathy, hypertrichosis, hyperpigmentation, scleroderma, and osteosclerotic plasmacytoma. POEMS syndrome
has been linked to infection with human herpes- virus type 8.
How Can You Di erentiate POEMS Syndrome from Mul-
tiple Myeloma with Di erent Body Manifestations ?
5 e osteolytic lesions of plasmacytoma and MM are
“purely” lytic, with punched-out appearance on skeletal
radiographs. In contrast, POEMS syndrome lesions are
seen as well-de ned u y sclerotic lesions or osteolytic
lesions with sclerotic margins.
5 Bone pain attributed to osteolytic lesions is common in
MM, whereas bone pain is unlikely to occur due to bony
lesions in POEMS syndrome.
5 Patients with MM are typically elderly, >60years of age,
while patients with POEMS syndrome are usually younger.
5 e monoclonal band in MM shows predominance of a
kappa light chain, while in POEMS syndrome, it is a
lambda light chain.
5 e presence of Bence Jones protein in the serum and/or
urine of classic MM patients is absent in POEMS
syndrome.
5 Bone scintigraphy is typically negative in MM, while it is
positive in POEMS syndrome.
outside the vertebra as a so -tissue mass arising from the
vertebral body.
Renal disease in multiple myeloma o en arises due to
amyloidosis, causing proteinuria in 60–90 % of cases and uremia in terminal stages, which is known as myeloma kidney .
Renal failure in MM patients commonly arises due to infections, calculi, or nephrocalcinosis, rather than the classic
myeloma kidney. In myeloma kidney, there is deposition of
an abnormal globulin of small molecular weight as droplets
in the cytoplasm of renal tubular epithelium. Later, brosis
and degeneration of the renal tubules occur, resulting in
replacement of the nephron by brous tissue. Identifying
Bence Jones protein in the urine is diagnostic of MM.Signs of
uremia in MM are similar to those of uremia due to other
causes, except that hypertension is rarely present. Intravenous
Signs on Plain Radiographs
5 The classical appearance of MM is that of punched-
out, sharply circumscribed, small osteolytic bone
lesion that can be solitary or multiple. The
osteolytic lesions are often found in the skull
(. Fig. 9.8.35 ), vertebrae, ribs, pelvis, and long
bones. Without the clinical picture, relying on
radiographs alone to diagnose MM is not always
possible, as multiple osteolytic bony lesions can be
also seen in metastatic cancer and other tumors.
5 Diffuse osteoporosis is commonly encountered in
MM.
urography should not be used in patients with MM to assess
9.8 · Multiple Myeloma (Kahler’s Disease)
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9
5 Pathologic fractures may be seen, especially in the
vertebrae.
5 Acute myeloma presents as multiple lytic lesions
(. Fig. 9.8.36 ), while chronic myeloma can present
as a dense and thick bone, mimicking Paget’s
disease.
5 Although multiple osteolytic lesions are found in
myeloma, bone scan is typically negative in MM.
5 POEMS syndrome lesions are seen as well-defined
fluffy sclerotic lesions or osteolytic lesions with
sclerotic margins.
5 Severe complication of di use in ltration of the
vertebral body includes vertebral collapse due to
pathological fracture. Severe collapse of the vertebral
body (vertebra plana) can be seen (. Fig. 9.8.37 ).
. Fig. 9.8.35 Lateral plain skull radiograph of a patient with
multiple myeloma shows multiple, osteolytic, sharply de ned
lesions a ecting the calvarium
. Fig. 9.8.37 Lateral plain thoracic vertebral radiograph of a
patient with multiple myeloma shows vertebra plana ( arrow )
Signs on CT
5 I n numb chin syndrome , there is an expansile bony
lytic lesion that destroys the mandibular ramus
and infiltrates the masticator space
(. Fig. 9.8.38 ).
5 Vertebral plasmacytoma is seen as an osteolytic
lesion with soft-tissue mass that grows externally
into the adjacent surrounding tissues
(. Fig. 9.8.39 ).
. Fig. 9.8.36 Anteroposterior plain radiograph of the right
hip joint shows di use small osteolytic lesions a ecting the
femur and the pelvis in a patient with acute multiple myeloma
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Chapter 9 · Hematology
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. Fig. 9.8.38 Axial upper jaw dental CT illustration of a
patient with numb chin syndrome due to multiple myeloma
shows soft-tissue mass destroying and violating the right
mandibular ramus integrity ( arrowhead )
of low T1 and high T2 signal intensities within the
vertebral bodies.
5 The presence of multiple scattered small foci
results in a variegated appearance (. Fig. 9.8.40 ).
The diffuse infiltration of the vertebral bodies
results in reducing the total signal intensity of the
vertebral column compared to the vertebral disks
on T1W images ( positive disk sign ). In the normal
vertebral MRI scan, the vertebral bodies have
higher signal intensity than the vertebral disks on
T1W images, due to the fatty bone marrow. The
positive disk sign is commonly also found in
patients with leukemia, when leukemic cells
diffusely infiltrate the vertebral column.
5 Infiltration of the meninges may be seen as
nodular or thickened meninges with contrast
enhancement ( Meningiosis carcinomatosis ).
5 The degree of vertebral body bone marrow
infiltration can be assessed by measuring the
enhancement difference. On T1W images, this is
done by applying a region of interest to the
vertebral body and measuring the signal intensity
(e.g., 240) and then copying the circle on the same
section that was measured and applying it to the
T1W postcontrast images to get the signal
intensity of the vertebral body postcontrast (e.g.,
320). Signal intensity (SI) difference is calculated
by the following formula: (SI after contrast– SI
before contrast/SI before contrast) × 100. Normal SI
signal difference should be <18 %. An SI difference
of >24 % reflects low-grade infiltration, while an SI
difference of >49 % reflects high-grade infiltration
(
. Fig. 9.8.41 ) .
. Fig. 9.8.39 Axial thoracic vertebral CT of a patient with
plasmacytoma shows osteolytic soft-tissue mass with external
and intraspinal canal extensions ( arrowhead )
Signs on MRI
5 The MR appearance of lesions of MM, often in the
vertebral column, is staged into four main types:
normal, focal, variegated, and diffuse. The normal
pattern shows no signs marrow infiltration of
vertebral bodies, which is a very good sign for
prognosis. The focal pattern shows localized areas
9.8 · Multiple Myeloma (Kahler’s Disease)
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. Fig. 9.8.40 Sagittal T1W thoracic vertebral MRI shows the
variegated appearance of multiple myeloma bone marrow
in ltration
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9
. Fig. 9.8.41 Sagittal T1W ( a ) and T1W postcontrast ( b ) of a patient with multiple myeloma shows two
circles that measure the signal intensity in the region of interest. By applying the formula, the signal intensity
di erence was 26 %, re ecting low-grade di use vertebral bone marrow in ltration
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Chapter 9 · Hematology
Further Reading
Angtuaco EJC, etal. Multiple myeloma: clinical review and
diagnostic imaging. Radiology. 2004;231:11–23.
Attwell A, etal. Multiple myeloma involving the porta hepatic
and peritoneum causing biliary obstruction and malignant ascites. Dig Dis Sci. 2005;50:1068–71.
Bauer A, etal. Neovascularization of bone marrow in patients
with multiple myeloma: a correlation study of magnetic
resonance imaging and histopathologic ndings. Cancer.
2004;101:2599–604.
Chong ST, etal. POEMS syndrome: radiographic appearance
with MRI correlation. Skeletal Radiol. 2006;35:690–5.
Eidner T, etal. Clinical manifestations of POEMS syndrome
with features of connective tissue disorders. Clin Rheumatol.
2001;20:70–2.
Hess T, etal. Atypical manifestations of multiple myeloma:
radiological appearances. Eur J Radiol. 2006;58:280–5.
Jacobs P.Myeloma Dis Mon. 1990;36:323–71.
Lecouvet FE, etal. Stage III multiple myeloma: clinical and
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prognostic value of spinal bone marrow MR imaging.
Radiology. 1998;209:653–60.
Leonard RCF, et al. Multiple myeloma: radiology or bone
scanning? Clin Radiol. 1981;32:291–5.
Libshitz HI, et al. Multiple myeloma: appearance at MR
imaging. Radiology. 1992;182:833–7.
Magnusson S, etal. Multiple myeloma. Dis Mon. 1960;6:1–32.
Narváez JA, etal. POEMS syndrome: unusual radiographic,
scintigraphic and CT features. Eur Radiol. 1988;8:134–6.
Patriarca F, etal. Meningeal and cerebral involvement in mul-
tiple myeloma patients. Ann Hematol. 2001;80:758–62.
Sugawara Y, etal. Paresthesia of the lower lip as a rst mani-
festation of multiple myeloma– a case report. Oral Radiol.
2003;19:158–66.
Winterbottom AP, et al. Imaging patients with myeloma.
Clin Radiol. 2009;64:1–11.
9.9 Amyloidosis
Amyloidosis is a systemic disease characterized by amyloid
protein depositions in the extracellular matrix components
such as blood vessel walls, the epithelial basement membrane,
and the connective tissue matrix.
Amyloid is a term used to describe any protein with a
“beta-pleated sheet” con guration. Amyloid proteins stain
brown with iodine stain, from which the name was derived
(amyloid means “starch-like”). Characteristically, amyloid
proteins stain dark red with Congo red stain. When viewed
under polarized light, amyloid stained with Congo red stain
displays an apple-green birefringence. Any brillar protein
with a “beta-pleated sheet” con guration will stain as amyloid.
Amyloid protein deposition in the extracellular matrix
causes thickening and narrowing of the small vessel walls,
destruction of the epithelial basement membrane, and mass
e ect over the cells, causing cellular ischemia and destruction over time. Any tissue can be a ected by amyloid deposition. Amyloidosis can be systemic, a ecting all body tissues,
or localized to a certain organ.
Classi cation ofAmyloidosis (Clinical-Based
Classi cation)
Systemic amyloidosis is a type of amyloidosis characterized by
widespread body tissue disease and amyloid presence in the
blood. e systemic form is divided into four major types:
5 B - cell dyscrasia ( primary amyloidosis ): this form arises
due to defect in the B-cell function. e B cells produce
amyloid precursor protein into the blood called
“light- chain amyloid” and referred to as amyloid (AL).
e monocytes engulf these AL amyloid precursors and
then resecrete them in the blood in the form of the
classic amyloid proteins. is type of amyloidosis is
typically seen in patients with multiple myeloma and
plasmacytoma (B-cell malignancies).
5 Reactive systemic amyloidosis ( secondary amyloidosis ):
this form arises due to abnormal chemical signaling that
evokes the liver to manufacture amyloid precursors and
secretes them into the blood. e abnormal signaling can
be initiated by di erent diseases. e liver forms
“amyloid- associated protein,” which is referred to as
amyloid (AA). Like amyloid AL, monocytes play a major
role in transforming amyloid AA precursor into complete
amyloid protein form. is type of amyloidosis can be
seen associated with diseases like ulcerative colitis,
Crohn’s disease, systemic vasculitis, tuberculosis,
Hodgkin’s disease, and rheumatoid arthritis.
5 Dialysis - associated amyloidosis : this is a special form of
systemic amyloidosis that occurs in patients on
hemodialysis (up to 70 % of cases). It is believed that this
form arises due to aggregation of β
within the ltration machine, which will form amyloid
protein, and then these amyloid proteins reenter the body
via the machine when the clear blood returns to the body.
is type of amyloid has an a nity to precipitate in the
joints, ligaments, tendons, and synovial membranes.
5 Hereditary familial amyloidosis : this form is rare, and it is
seen in families and rare syndromes. An example of
hereditary amyloidosis is Muckle – Wells syndrome , which is
a rare autosomal dominant disease characterized by
chronic recurrent urticaria, o en combined with fever,
chills, rigors, arthralgia, progressive sensorineural hearing
loss, and AA-type amyloidosis in 30 % of cases. Another
example of hereditary amyloidosis is familial
Mediterranean fever. Familial Mediterranean fever ( Familial
paroxysmal polyserositis ) is a genetic disease with autosomal
recessive mode of inheritance, characterized by episodes of
fever, abdominal pain, arthritis, and amyloidosis. e
disease is common among Iraqi Jews, Armenians, Turks,
and Middle Eastern Arabs. Patients experience multiple
attacks of fever that last 12–72h and resolve spontaneously.
Recurrent attacks of abdominal pain that mimics acute
abdomen are common, with constipation and diarrhea. e
abdominal attack typically improves spontaneously in
24–72h. Arthritis, including large-joint mono- and
polyarthritis, is a common feature. Seronegative HLA-B27
sacroiliitis and ankylosing spondylitis are reported among
patients with familial Mediterranean fever.
-microglobulins
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9.9 · Amyloidosis
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k Localized Amyloidosis
is type of amyloidosis is characterized by deposition in a
speci c tissue (e.g., renal parenchyma). In this type, the amyloid proteins are manufactured in the a ected tissue.
Examples of localized amyloidosis include:
5 Amyloidoma : this is a rare form of deposition of amyloid
in a certain tissue, forming a solid mass in the absence of
B-cell disease (dyscrasia) or elevation of serum proteins.
Amyloidoma can occur in any body tissue and cannot be
di erentiated from other tumors except by biopsy.
5 Hormonal amyloidosis : an example of this type is seen in
endocrine cancerous cells that secrete amyloid proteins
rather than normal hormones (e.g., thyroid medullary
carcinoma).
5 Senile amyloidosis : deposition of amyloid proteins due to
the aging process in the choroids plexus, brain, and heart.
Up to 30 % of patients with B-cell dyscrasia progress to
multiple myeloma, while multiple myeloma is associated
with systemic amyloidosis in 15 % of cases. e median survival rate in patients with AL-type amyloidosis is 1.5years,
whereas the median survival rate in patients with AA-type
amyloidosis is 4.5years.
Although the features of amyloidosis are not speci c,
radiologists need to be familiar with the disease manifestations in di erent body organs, especially in secondary amyloidosis. Secondary amyloidosis can be suspected in patients
with systemic diseases that present with body manifestations
that cannot be explained by the original disease symptoms.
Renal amyloidosis can be divided into early and late stages. In
the early stage, the kidney is normal in size and shape, a er which
it starts to progressively increase in size, due to the amyloid
deposition. e enlarged amyloid kidney is rm in consistency
and has a waxy appearance on postmortem gross examination.
In later stages, chronic parenchymal ischemia occurs due to
amyloid deposition within the renal vessels, which causes irreversible cell damage and brosis. e end result of renal amyloidosis is renal failure. Patients with kidney amyloidosis commonly
present with nephrotic syndrome , a syndrome characterized by
generalized edema, hyperlipidemia, hematuria, and gross proteinuria (>3g/L). Bladder amyloidosis is o en seen as a solitary
mass (amyloidoma), which presents clinically with hematuria.
Hepatic amyloidosis can occur, but usually does not prog-
ress into liver failure. Normally, the liver parenchymal reservation is 85 % of its mass, and the renal parenchymal reservation
is 75 % of the kidneys’ mass. Due to these facts, most patients
with systemic amyloidosis rarely develop hepatic failure,
because they may die from renal failure before developing complete hepatic failure. However, hepatic dysfunction is observed,
but hepatic failure is rare. e amyloid proteins are deposited in
the arterioles, the extracellular compartments, and the hepatic
sinusoids (space of Disse) until they ll the sinusoids and exert
back pressure on the hepatocytes, causing pressure atrophy.
Splenic amyloidosis is detected clinically in the form of
splenomegaly. e spleen is made of white pulp (15 %) and
red pulp (85 %). Amyloidosis of the spleen may a ect the
white pulp or the red pulp. When amyloidosis a ects the
white pulp, it results in a moderately enlarged spleen, with a
patchy, waxy appearance in postmortem gross examination
(sago spleen). When it a ects the red pulp, it causes di use
enlargement, with di use waxy appearance in postmortem
gross examination (di use amyloid spleen).
Cardiac amyloidosis is generally a rare condition. It can
arise due to senility or due to chronic systemic disease.
Amyloidosis of the heart can a ect the atria more than the
ventricles, for unknown reasons, and it may cause restrictive
cardiomyopathy. Cardiac amyloidosis is usually caused by
AL-type amyloidosis and rarely by AA-type amyloidosis.
Endocrine amyloidosis may occur and is classically seen in
the form of endocrine insu ciency of the pituitary gland (hypopituitarism) or adrenal gland insu ciency (Addison’s disease).
Gastrointestinal tract amyloidosis is detected as a disease
of hollow organs. e colon is the most frequently a ected
organ. In the intestine, amyloid accumulates within the arterioles of the intestinal villi, resulting in malabsorption and
diarrhea (due to failure of the villi to function), and mucosal
ulceration and bleeding (due to villi ischemia and necrosis).
Esophageal and gastric involvement results in dysmotility,
wall thickening, and gastroesophageal re ux disease.
Pulmonary amyloidosis is a relatively rare condition, with
patients o en presenting with recurrent pneumonias, which
characteristically occur in the same distribution that correspond to previous antibiotic treatment, but recurs at a later
time. Features of pulmonary amyloidosis include di use
interstitial nodular pattern, tracheal and bronchial wall
thickening, and (rarely) a solitary mass (amyloidoma).
Central nervous system amyloidosis is o en present in the
form of cerebral amyloid angiopathy (CAA) with spontaneous nontraumatic intracranial bleeding or (rarely) as leptomeningeal thickening.
Musculoskeletal amyloidosis generally causes muscular
hypertrophy, weakness, and chronic pain. Muscular amyloidosis preferentially involves the shoulder girdle. Deposition
of amyloids within the periarticular tissues of the shoulder
girdle resulting in shoulder enlargement is called the “shoulder pad sign” (
. Fig. 9.9.42 An illustration demonstrates the shoulder pad sign
(right shoulder)
. Fig. 9.9.42 ).
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