1

DISORDERS
OF
LYMPHORETICULAR
SYSTEM
 FOUR MAIN GROUPS OF DISORDERS 2

A. Lymphomas: Including Hodgkin's and non-Hodgkin's lymphomas

B. Plasma-cell disease: Plasmacytoma and multiple myeloma
C. Histiocytosis
D. Storage disorders: Gaucher's disease and Niemann-Pick disease.
 3

HODGKIN'S AND
NON-HODGKIN'S LYMPHOMA
 4

LYMPHOMA
A group of malignant tumors originated from
lymph nodes or other lymphatic tissues( spleen,
bone marrow , GIT, liver etc)

Malignant tumors of immune system

 CLASSIFICATION OF 5

LYMPHOMA

 Hodgkin Disease (HD/HL)

 Non-Hodgkin Lymphoma (NHL)

 6

INCIDENCE
• 50% at age 20~40
• Male : Female 1.4~3.7:1
• NHL 90%
• HD 10% (more frequent in western countries)
• In children, lymphoma is the third most frequent
malignancy, following behind leukaemia and central
nervous system neoplasia.
 7

General symptoms:

I. Fever
II.Weight loss
III.Loss of appetite
IV.Night sweats
V.Pruritus
VI.lethargy
 SIGNS
8

1.Lymph node enlargement

2.Cachexia
3.Anemia
4.Splenomegaly
5.Hepatomegaly
6.Jaundice rarely
 9

HL NHL

A single axial LN is usually Multiple peripheral LN,

involved mesenteric nodes & waldeyer’s
( cervical, mediastinal , para- ring are usually involved.
aortic) mesenteric & waldeyer’s
ring rarely involved.

Spreads by contiguity Noncontiguous spread

Extranodal involvement is Common

uncommon

RS(Reed-strernberg) giant cells Absent

are always present
 10

REED STRENBERG CELL

• Criteria:
1. It is a malignant giant cell
2. Large cell with paired mirror
image nuclei.
3. This nuclei resembles owl’s
eye appearance.
 11

PATHOLOGICAL CLASSIFICATION - HL
Nodular sclerosis---Relatively favorable prognosis.
Mixed cellular---Both lymphocytes & histeocytes are
present ,tendency of dissemination, relatively poor
prognosis.
Lymphocyte predominant---There is heavy
lymphocytic infiltration of the node, RS cells are few,
good prognosis.
Lymphocyte depleted--- Lymphocytes are small &
large number of RS cells, poor prognosis.
 12

CLASSIFICATION OF NHL
According to histological architecture:
1. Follicular and
2. Diffuse.
As a working formulation the tumour is classified in to:
1. Low grade.
2. Medium grade.
3. High grade
 CLINICAL STAGING OF HL
& NHL (ANN ARBOR)
• Stage I
Involvement of a
single lymph node
region or of a
single extranodal
organ or site.

13
 CLINICAL STAGING (ANN
ARBOR )
• Stage II:
• two or more
lymph node
regions on the
same side of the
diaphragm
• localized
extranodal organ
and one or more
lymph node
regions on the
same side of the
diaphragm

14
 CLINICAL STAGING (ANN
ARBOR)
• Stage III

lymph node regions on

both sides of the
diaphragm or with
localized extranodal
organ (III E) or spleen
(IIIs)or both (III ES).

15
 CLINICAL STAGING (ANN
ARBOR)
• Stage IV:
Diffused or
disseminated
involvement of
one or more
extranodal
organs, with or
without
associated
lymph node
enlargement.

16
 R/F 17

• Nodal disease:
1. Thorax: Intrathorasic nodal
disease is more common in HD
than NHL. Between 67% to 85% of
patient with HD have mediastinal
involvement at the time of
presentation compared with 40% to
50% of patient with NHL.

PA and lateral radiograph of the chest shows

mediastinal adenopathy
(red arrows) producing lobulated soft tissue
masses
 18

The chest film shows the typical features of Hodgkin lymphoma, e.g., an
anterior mediastinal mass.
 19

There is an obvious large mediastinal mass on

the right at the level of the mid-lung. It is
homogenous and appears to be a solid mass.
On the CT images, it is of a soft tissue density,
suggesting lymphoid tissue.
 20

A large mediastinal nodal mass in a patient presenting with Hodgkin's disease

is seen compressing the major airways.
 21

Chest X-ray in another patient with

Hodgkin’s disease - showing enlarged
mediastinal lymph nodes and infection in
lung bases
 22

PA and lateral chest X-ray: A conglomerate of enlarged lymph nodes

can be seen forming a sharp-contoured shadow which widens the
mediastinum on the right (arrow).
 23

Hodgkin lymphoma.
Axial CT show an anterior mediastinal mass and paratracheal
adenopathy.
 ABDOMEN & PELVIS 24

• CT section showing mesenteric lymph node involvement

(arrowheads) in a patient with non-Hodgkin's lymphoma. This is
common in non-Hodgkin's lymphoma, but seen in fewer than 5%
of patients with Hodgkin's disease.
 25

CT section through the pelvis showing bilateral pelvic and groin

lymph node enlargement in a patient with non-Hodgkin lymphoma.
 26

The lymph nodes are generally

enlarged and appear foamy in a
patient with lymphoma.
 27

EXTRANODAL
PRESENTATION OF THE
NHLS
 GASTROINTESTI 28

NAL LYMPHOMA

CT scans through the abdomen of a patient

with gastric NHL showing thickening of
the gastric wall and prominence of the
rugal folds, with associated nodal disease,
(A) before (B) and after treatment.
 29

THORACIC LYMPHOMA
• Involvement of lung is a
common feature of both HL
& NHL
• In high grade NHL
pulmonary involvement may
be extensive & explosive

CT scan showing pulmonary involvement in a

patient with NHL. A mass of tumour tissue is
seen around the right hilum. In addition, a
number of ill-defined intrapulmonary nodules
are seen on the right.
 30

Adult T-cell
lymphoma presenting
with liver and spleen
disease. Although in
this case the use of
intravenous contrast
demonstrated splenic
involvement it did not
change patient
management.
 31

CT scan on a patient with NHL shows

enlargement of the retrocrural lymph
nodes (arrowhead). A = aorta.
 IN THE 32

SKELETON
• Destructive lesion either
due to direct infiltration
of bone or by
involvement from
affected lymph node in
red marrow area.
• Sternum: osteolytic
lesion may involve with
presternal and
retrosternal soft tissue
swelling.
Vertebra:
33
1. Anterior erosion of
vertebral body, with or
without reactive sclerosis.
2. Solitery dense vertebra
suggestive of this disease.
3. Multiple vertbra may be
involved.
4. Preservation of vertebral
endplate density.

Hodgkin's disease. (A) The common pattern of

endosteal sclerosis and patchy bone destruction
is shown in the vertebral body of T9 in an adult
man. Similar changes are also present at TI 1 .
These features are virtually diagnostic.
 34

Fig. 41.31 (A,B) Non-

Hodgkin's lymphoma.
pathological
fracture of a lumbar vertebral
Body.
 35

Hodgkin's disease. CECT section of the abdomen demonstrates a densely sclerotic lumbar
vertebral body associated with a large paravertebral soft-tissue mass.
 36

Fig. saittal (C) and coronal (D) T1-

weighted images show low signal intensity within the upper lumbar vertebral body due to diffuse
sclerosis and the extent of the soft-tissue mass. This
extends into the central spinal canal and circumferentially around the thecal sac.
 37

Fig. 41.26 Non-Hodgkin's lymphoma. A conventional radiograph (A) is virtually

normal save for the slight suggestion of patchy ill-defined bone destruction.
Subsequent T1,-weighted coronal (B) MR images demonstrate not only extensive
marrow replacement but also a substantial enveloping
soft-tissue mass.
 BURKITT’S LYMPHOMA 38

• An exceptional and particularly aggressive

form of non-Hodgkin's lymphoma is
common in African children.
• Large, destructive lesions develop
especially in the mandible and maxilla.
• Radiologically these lesions are purely
osteolytic and grow rapidly.
• The disease is associated with a virus
(Epstein-Barr).
 39

BURKITT’S
• The jaw lesions are LYMPHOMA
characterised by the
resorption of the
lamina dura with
multiple lytic foci that
eventually coalesce
with radiating spicules
of bone.
 BURKITT’S LYMPHOMA 40

• An 8-year-old boy with Burkitt's lymphoma and diffuse marrow involvement.

• Fat-suppressed, T2-weighted sagittal MR image of the lumbar spine shows
heterogeneous signal intensity of the bone marrow and epidural extension of tumor
(arrowheads) at the level of L5 and L2, with apparent preservation of the vertebral
cortex.
 DISORDERS OF LYMPHORETICULAR 41

SYSTEM
A. Lymphomas: Including Hodgkin's and non-Hodgkin's lymphomas
B. Plasma-cell disease: Plasmacytoma and multiple myeloma
C. Histiocytosis
D. Storage disorders: Gaucher's disease and Niemann-Pick disease.
 42

PLASMA CELL DISORDER

• Plasma cells represent the end product of B-lymphocyte maturation.
Pathological proliferation produces either a local tumour
(plasnmacytoma) or disseminated disease(Multiple myelomatosis).
• Types:
1.Plasmacytoma
2.Multiple myelomatosis.
 43

PLASMACYTOMA
• This condition is unifocal, causing a localised destructive lesion in
the skeleton, in a red marrow area.

C/F: bone pain, particularly in thoracolumber and lumber region.

Ilium, femur, humerus are next most commonly involved area.

Age: 30-60 yrs.

 PLASMACYTOMA 44

• R/F:
1.These lesions arises in area of red
marrow function with thin
overlying cortex.
2. Coarse trabecular strands of
increased density may give a
network appearance in the area of
destruction, and exceptionally the
lesion may be entirely sclerotic.
Large lesions in flat bones may
assume a soap-bubble appearance.

Fig. 41.36 Plasmacytoma of pelvis. Bone

expansion is associated with
coarse trabeculation, producing a soap-bubble
appearance.
 45

MULTIPLE
MYELOMATOSIS
• The disseminated or generalised form of plasma cell infiltration of
bone marrow is known as multiple myelomatosis.
• Age: over 40.
• Sex: M:F is 2:1.
• C/F: Persistent bone pain or a pathological fracture are usually the
first complaints.
 46

MULTIPLE MYELOMATOSIS.
• Plasma cell proliferation causes production of abnormal
immunoglobulins. Such proliferation eventually takes place at the
expense of all other marrow functions so that a non-specific
Icukopenia and secondary anaemia develop.
• presence of an abnormal urinary protein constituent, Bcnce Jones
proteose, may be demonstrated.
• Hypercalcaemia, Hypercalcuria and Amyloidosis may occur.
• R/F:
1. Axial skelitons are affected 47

predominently Lesions may be

observed also in the shafts of long
bones and in the skull.
2. Lesions are osteolytic with no bony
reaction.
3. generalised reduction in bone
density and localised areas of
radiolucency in red marrow areas.

Fig. 41.40 Myelomatosis. Diffuse

marrow involvement has resulted in an
overall reduction in bone density
 MULTIPLE MYELOMATOSIS 48

• R/F:
• The rounded and oval defects
that develop are characterised
by the sharp definition of their
edges. Reactive marginal
sclerosis is absent.
• Pathological frature may
present.

Fig: Typical localised lesion of

myeloma are demonstrated in the upper
femur. sharply defined rounded defects
with endosteal erosion.
 49

• Vertebra: MULTIPLE
1. overall reduction in bone density MYELOMATOSIS
with osteoporotic spine, may
accompanied with collapsed vertebral
body.
2. para vertebral soft tissue shadow
are common. Intervertebral disc space
and articular surface are not affected.
 DISORDERS OF LYMPHORETICULAR 50

SYSTEM
A. Lymphomas: Including Hodgkin's and non-Hodgkin's lymphomas
B. Plasma-cell disease: Plasmacytoma and multiple myeloma
C. Histiocytosis
D. Storage disorders: Gaucher's disease and Niemann-Pick disease.
 51

HISTIOCYTOSIS
• Pathological proliferation of histiocytic cells occurring
particularly in the bone marrow, the spleen, the liver,
the lymphatic glands and the lungs.
• In more chronic form these cells become swollen with
lipid deposits, essentially cholesterol and they present
the pathological appearance of Foam cells.
• Some of these become necrotic and replaced by fibrous
tissue.
 52

HISTIOCYTOSIS
• It includes:
1. Eosinophilic Granuloma
2. Hand Schuller Christian Disease
3. Letterer Siwe Disease
 53

EOSINOPHILIC
GRANULOMA
• Histology: Eosinophilic infiltration around the collection of
histiocytes. Mild necrosis and fibrosis rarely present. Appearance
of foam cells suggest more serious variety. confirmatory biopsy is
usually unnecessary.
• Age: children especially boys, between 3 and 12 years are most
commonly affected.
• C/F: pain, swelling, mild fever.
 54

EOSINOPHILIC
• R/F:1. Translucent GRANULOMA
areas of bone
destruction, with
sharply defined margins
and often of
considerable size, are
characteristic. The
round or oval defects
may have scalloped
margins Fig. Histiocytosis. A purely osteolytic lesion
is present in the mandible, with well-defined,
slightly scalloped margins. The lamina dura has
been destroyed. The teeth seem to 'float in air'.
 EOSINOPHILIC GRANULOMA 55

• R/F :
2. vertebra: Solitary
lesions in the spine
may collapse, partially
or completely the latter
presenting the classic
appearance of vertebral
plana. The most
commonly affected site
is the thoracic spine.

Fig. 41.45 Histiocytosis. Vertebral lesions in the

thoracic spine shown on a lateral tomogram. The
bodies of T7 and 8 have collapsed with a slight
increase in bone density. Note the relative
preservation of the disc spaces.
 EOSINOPHILIC GRANULOMA 56

R/F :
• The walls of the
affected vertebral
body tend to bulge
laterally.
• The disc spaces on
either side remain
intact and may appear
widened.
• paravertebral soft-
tissue shadows may
be evident.

Fig. 41.45 Histiocytosis. Vertebral lesions in

the thoracic spine shown on a lateral view. The T1W Image:Eosinophilic
bodies of T7 and 8 have collapsed with a slight granuloma of the vertebral
increase in bone density. Note the relative body
preservation of the disc spaces
 EOSINOPHILIC GRANULOMA 57

• Severe loss of T3 vertebral body height (i.e. vertebral plana) is present

with preservation of the adjacent disk space. The affected vertebral body
demonstrates low signal intensity on T1WI and slightly increased signal
intensity on T2WI compared to the normal bone marrow. Contrast-
enhanced MR imaging demonstrates associated enhancement of the
vertebral lesion and of the
 EOSINOPHILIC GRANULOMA 58

R/F :
• This benign form of
histiocytosis occasionally
affects long bones and
initially has a predilection
for the
diaphysis.However,
metaphyseal involvement
may occur.

Fig. 41.46 Histiocytosis. A healing diaphyseal

lesion exhibits periosteal new bone formation
and minimal sclerosis around the margins of
the radiolucency.
 EOSINOPHILIC GRANULOMA 59

Fig. 41.49 Histiocytosis. Adult pulmonary involvement (man aged 20). (A) A localised view from a chest
radiograph demonstrates a coarse interstitial pulmonary fibrosis. Note also a pathological fracture of the
left fourth rib due to a bony deposit. (B) A CT scan demonstrates peripheral interstitial pulmonary fibrosis
with thickened interlobular septa and irregular honeycombing.
EOSINOPHILIC GRANULOMA 60

Fig. 41.44 Histiocytosis. Extensive

involvement of a bone, here the clavicle,
is often associated with layered
periosteal new bone causing bony
expansion.
 61

HAND SCHULLER
• This lateral CHRISTIAN DISEASE
radiograph of the
skull demonstrates
the classic
geographic lytic
findings of a low-
grade inflammatory
process, such as
Hand-Schüller-
Christian disease
 62

HAND SCHULLER
• This is a more chronic form of the disease, with dissemination of
CHRISTIAN DISEASE
lesions in the lungs, lymph nodes, liver and spleen, in addition to
virtually constant and early involvement of the skeleton.
• Age: Children below 5 yrs.
 63

HAND SCHULLER
CHRISTIAN DISEASE
• C/F:
1.It is characterised by soreness of the mouth and loose teeth,
dueto deposits in the gums and jaws, and skin lesions.
2. Abnormalities in the temporal bone are common, with an
associated otitis media.
 HAND SCHULLER CHRISTIAN 64

DISEASE
• R/F:
1.The bone defects are
essentially the same as those
of eosinophilic
granulomas.They are far
more numerous and
particularly affect the flat
bones.
• 2. In the skull widespread
irregular defects usually
likened to a map and
described as the `geo-
graphical skull‘. both tables Fig: lateral radiograph of the skull
often being involved. demonstrates the classic geographic lytic
findings of a low-grade inflammatory process,
such as Hand-Schüller-Christian disease
 65

HAND SCHULLER
CHRISTIAN DISEASE
 HAND SCHULLER CHRISTIAN
66

DISEASE
• R/F:
2.Lesions i n the mandible
and maxilla begin around
the tooth roots, teeth, which
are never affected, remain
dense and appear to 'float in
o that the air.

Fiig: Hand-Schuller-Christian
disease
6 year-old, male
Panoramic /PA/Lateral view.
 67

HAND SCHULLER CHRISTIAN DISEASE

• R/F: The lungs

show a fine nodular
infiltration in the
acute phase.
 68

• R/F:
HAND SCHULLER
1. Several vertebrae may be CHRISTIAN DISEASE
completely or partially collapsed
and extensive lesions may develop
in the scapulae, ribs and pelvis.
Osteolytic pelvic lesions or
vertebral collapse in a child are
suggestive of this disease.
 69

LETTERER SIWE DISEASE

• This is an acute or subacute disseminated form of the disease.
• Age: occurring very rarely in infants below the age of 2 years.
• C/F:
1.Pyrexia, with a rash
2.Bmouth sores,
3.Bleeding gums,
4.Respiratory symptoms
5. Failure to thrive
6. Enlargement of liver, spleen, lymphnodes.
 LETTERER SIWE DISEASE 70

R/F:
1.Widely spread both in
the flat bones and
especially in the
metadiaphyseal areas of
the long bones.
2. Diffuse pulmonary
infiltration is
commonis a variety of
honeycomh lung.

Fig. 41.50 Histiocytosis: Letterer-Siwe type. Massive destructive lesions

are present throughout the skeleton, but affect particularly the metadiaphyseal
areas of the long bones
 DISORDERS OF LYMPHORETICULAR 71

SYSTEM
A. Lymphomas: Including Hodgkin's and non-Hodgkin's lymphomas
B. Plasma-cell disease: Plasmacytoma and multiple myeloma
C. Histiocytosis
D. Storages disorders: Gaucher's disease and Niemann-Pick disease.
 72

Storage disorders:
• Gaucher's disease and
• Niemann-Pick disease.
 73

GAUCHER'S DISEASE
• Gaucher disease (GD) is the most common lysosomal storage
disease in humans caused by deficiency of Beta-glucosidase.
C/F:
1. weakness and fatigue with progressive dementia.
2.Bone pain may be present, sometimes severe.
Clinical examination :
Splenic enlargement is detected in 95%of cases.
 74

GAUCHER'S DISEASE
• Histology:
1. Numerous large histiocytes within which an abnormal
lipoprotein, kerasin, is present.
2.These cells are disseminated throughout the marrow of the
haematopoietic skeleton,in addition to the spleen and liver.
 75

GAUCHER'S DISEASE
Skeletal involvement is seen in 70 -
100% of patients primarily
involves long bones (tibia,
humerus, femur) as well as
vertebrae. Ribs, hands and wrists,
ankles and feet, and mandible may
also be involved
Plain film
1. osteopenia
2. osteonecrosis
3. pathological / crush fractures
4. endosteal scalloping
5. Erlenmeyer flask deformities
6. H shaped vertabrae
 76

GAUCHER'S DISEASE
• H-shaped vertebra on frontal
view- due to avascular
necrosis of endplates-- seen
in Gaucher’s diaease.
 77

GAUCHER'S DISEASE
H-shaped vertebra:
normal vertebral body
height is maintained
anteriorly, there is a very
abrupt loss of height in
the midportion
 GAUCHER'S DISEASE 78

Fig. 41.53 Gaucher's disease-acute bone infarction. (A) A radiograph of a 1 3-year-old

girl, with known Gaucher's disease, presenting with acute hip
pain of 12 hours' duration. Slight endosteal sclerosis is shown in the inferior pubic ramus and
an area of ill-defined radiolucency in the intertrochanteric
region.
 GAUCHER'S DISEASE 79

Fig. 41.54 Gaucher's disease. This adolescent has considerable deformity

of the femoral head and acetabulum secondary to episodes of infarction.
 80

• Erlenmeyer flask deformity differential

•
• hemglobinopathies
• anemia
• fibrous dysplasia
• Gaucher’s disease
• Niemann Pick’s disease
• osteopetrosis
• craniometaphyseal dysplasia
• heavy metal poisoning
 81

NIEMANN-PICK DISEASE
• Niemann-Pick Disease is one of a group of lysosomal
storage diseases that affect metabolism and that are caused by
genetic mutations(acid sphingomayalinase deficiency).
• Rare disorder of the lymphoreticular system, similar to Gaucher's
disease.
• Osseous changes are less severe than in Gaucher's disease.
• Generalised osteoporosis, minor coarsening of the trabeculae and
minor modelling abnormalities are seen.
 82

• H-shaped vertebra (also known as Lincoln log vertebra) are a

characteristic finding of sharply delimited central endplate
depression classically seen in approximately 10% of patients with
sickle-cell anaemia and results from microvascular endplate
infarction 3.
 83

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