Conditions of the Lymph System

Anatomy of head & neck lymph nodes

Anatomy of axillary lymph nodes
Anatomy of inguinal lymph nodes
Generalized adenopathy has been defined
as
involvement of three or more
noncontiguous lymph node areas.
 CAUSES OF LYMPHADENOPATHY
Infection
• Bacterial (e.g., all pyogenic bacteria, cat-scratch disease, syphilis)
• Mycobacterial (e.g., tuberculosis, leprosy)
• Fungal (e.g., histoplasmosis )
• Chlamydial (e.g., lymphogranuloma venereum)
• Parasitic (e.g., toxoplasmosis, filariasis)
• Viral (e.g., Epstein-Barr virus, cytomegalovirus, rubella, HIV)
Benign disorders of the immune system (e.g., rheumatoid arthritis, SLE)
Malignant disorders of the immune system (e.g., chronic and acute myeloid and
lymphoid leukemia, non-Hodgkin's lymphoma, Hodgkin's disease,
angioimmunoblastic-like T-cell lymphoma, multiple myeloma with amyloidosis,
malignant histiocytosis)
Other malignancies (e.g., breast carcinoma, lung carcinoma, melanoma, head and
neck cancer, gastrointestinal malignancies, germ cell tumors, Kaposi's
sarcoma)
Storage diseases (e.g., Gaucher's disease)
 Acute lymphadenitis
• It is painful
• Localizing symptoms suggest infection in
specific site( primary focus )
• Constitutional symptoms may be present
e.g. Fever, malaise
• Treatment is directed to the cause
,antibiotics, derange if abscess occur
 Chronic non specific lymphadenitis
• Mild recurrent lymphadenitis
• Common sites are upper deep cervical LN
and inguinal
• Chronic enlargement of affected LN, which
is firm slightly tender and mobile
• Primary focus is there
• Treatment is directed to the cause
 Specific lymphadenitis

• Bacterial (cat-scratch disease, syphilis)

• Mycobacterial (e.g., tuberculosis, leprosy)
• Fungal (e.g., histoplasmosis,
coccidioidomycosis)
• Chlamydial (e.g., lymphogranuloma
venereum)
• Parasitic (e.g., toxoplasmosis, filariasis)
• Viral (e.g., Epstein-Barr virus,
cytomegalovirus, HIV)
 HIV- related.Persistent
Generalized Lymphadenopathy
(PGL)
Lymph nodes larger than 1.5 cm in diameter in 2 or more extrainguinal sites
of 3 or more months duration

Nodes are non-tender, symmetrical, and often involve the posterior cervical,
axillary, occipital, and epitrochlear nodes
Develops in up to 50% of HIV-infected individuals
Up to one-third do not have any other symptom on presentation (stage 1)
In HIV-positive patients, PGL is a clinical diagnosis.
 PGL may slowly regress during the course of HIV infection and may disappear
before the onset of AIDS
 Tuberculosis lymphadenopathy
• Cervical nodes most commonly involved
• Organism
• Root : lymph Vs blood
• Predisposing factors are immunocompromised patients
• Usual course of lymph born disease is as follows:

Firm, discrete nodes→Matted together and adherent to the surrounding →Fluctuant nodes , non tender abscesses,
chronic sinuses ( with dusky red skin over and undermined edge )

Anorexia ,night fever, night sweating, loss of weight

Fluctuant cervical nodes that develop over weeks to months without significant inflammation or tenderness suggest
infection with M. tuberculosis, atypical mycobacterium, or scratch disease
Blood born usually occur in adult with more than one group of LN which are firm, mobile and discrete
• CBC,CXR and positive smears for acid-fast bacilli
PCR
• Fine-needle aspiration of the involved lymph node spiral
In smear-negative pulmonary TB, it is worthwhile aspirating extra-thoracic lymph nodes to confirm diagnosis of TB
(80% positive)
Treatment
Anti tuberclus drugs, good diet, correct immune deficiency
Aspiration of abscess
•
Lymphoma
Lymphoma is a malignant disease that affects blood cells called
lymphocytes – immune cells that normally protect you from illness.
• Damage to genes in these cells can sometimes lead to abnormal cell
behavior which makes the cells immortal – unable to die when they should – or
causes sustained rapid cell division..
• These malignant cells then may accumulate to form tumors that enlarge the
lymph nodes or spread to other areas of the lymphatic system, such as the
spleen or bone marrow, or outside the lymphatic system to the skin, or mucosal
linings of the stomach.
• They arise as the result of abnormal proliferation of the lymphoid system, and
hence occur at any site where lymphoid tissue is found. Most commonly they
are manifest by the development of lymphadenopathy at single or multiple
sites, although primary extranodal presentations account for up to 20% of non-
Hodgkin's lymphoma.
• The prognosis is determined by the specific subtype of lymphoma and the
anatomical extent of disease and its bulk, the clinical course ranging from
months to years.
• Lymphomas are currently classified on the basis of histological appearance
into:
Hodgkin's lymphoma
non-Hodgkin's lymphoma.
The two types not only have different morphologic characteristics but differ also in
their clinical behavior and their response to various therapeutic regimens.
HODGKIN'S LYMPHOMA (HL)
• Aetiology
There is epidemiological evidence linking previous infective mononucleosis with
HL and up to 40% of patients with HL have increased EBV antibody titres at the
time of diagnosis and several years prior to the clinical development of HL.
Other environmental and occupational exposure to pathogens have been
postulated.
?? Chronic infection,?? Depressed immunity,?? Chemical exposure – pesticides,cancer
therapies, herbicides ,?? Viral exposures
• Pathology :
The hallmark of HL is the Reed-Sternberg cell) which is usually derived from
germinal centre B cells or, rarely, peripheral T cells.
• Pathological classification of Hodgkin's lymphoma
Nodular lymphocyte-predominant Hodgkin's lymphoma
Classical Hodgkin's lymphoma  
Nodular sclerosis HL(young females, involving particularly lymph nodes in
the mediastinum and neck ).
  Lymphocyte-rich HL(It often occurs in peripheral lymph nodes. It is often an
indolent disease(.  
  Mixed cellularity HL) more common in men and is associated with B
symptoms ( 
  Lymphocyte-depleted HL) It is seen in HL associated with HIV (
 Clinical features
• Lymph node enlargement, most often of the cervical
nodes (other causes are shown in, these are usually
painless and with a rubbery consistency.
• Enlargement of the spleen/liver.
• 'B' symptoms: fever, (25%) drenching night sweats,
weight loss of > 10% bodyweight
• Other constitutional symptoms, such as pruritus, fatigue,
anorexia and, occasionally, alcohol-induced pain at the
site of enlarged lymph nodes.
• Symptoms due to involvement of other organs (e.g. lung
- cough and breathlessness (
 Investigations
• Blood count may be normal, or there can be a normochromic,
normocytic anaemia. Lymphopenia and occasionally eosinophilia
are present.
• Erythrocyte sedimentation rate )ESR) is usually raised and is an
indicator of disease activity.
• Liver biochemistry is often abnormal, with or without liver
involvement.
• Serum lactate dehydrogenase; raised level is adverse prognostic
factor.
• Chest X-ray may show mediastinal widening, with or without lung
involvement.
• CT scans show involvement of intrathoracic nodes in 70% of cases.
Abdominal or pelvic lymph nodes are also found. It is the
investigation of choice for staging although PET scanning is
increasingly being used.
• Lymph node biopsy is required for a definitive diagnosis
 Cotswolds modification of Ann Arbor
staging classification
Stage I Involvement of a single lymph-node region or lymphoid structure (e.g. spleen, thymus,
Waldeyer's ring) or involvement of a single extralymphatic site
Stage II Involvement of two or more lymph-node regions on the same side of the diaphragm (hilar
nodes, when involved on both sides, constitute stage II disease); localized contiguous
involvement of only one extranodal organ or site and lymph-node region(s) on the same side of
the diaphragm (IIE). The number of anatomic regions involved should be indicated by a
subscript (e.g. II3)
Stage III Involvement of lymph-node regions on both sides of the diaphragm (III), which may also be
accompanied by involvement of the spleen (IIIS) or by localized involvement of only one
extranodal organ site (IIIE) or both (IIISE)III1With or without involvement of splenic, hilar, coeliac,
or portal nodesIII2With involvement of para-aortic, iliac, and mesenteric nodes
Stage IV Diffuse or disseminated involvement of one or more extranodal organs or tissues, with or
without associated lymph-node involvement
Designations applicable to any disease state
A No symptoms
B Fever (temperature > 38°C), drenching night sweats, unexplained loss of more than 10% of
body weight within the previous 6 months
X Bulky disease (a widening of the mediastinum by more than one-third of the presence of a
nodal mass with a maximal dimension greater than 10 cm)
E Involvement of a singe extranodal site that is contiguous or proximal to the known nodal site
 Treatment
• Specific treatment is based otherwise on the
anatomical distribution of disease, its 'bulk' and
the presence or absence of 'B' symptoms
• 'Early stage' (IA, IIA no bulk): The treatment of
choice now is brief chemotherapy followed by
involved field irradiation.
• Advanced disease :Cyclical combination
chemotherapy with or without irradiation to sites
of 'bulk' disease is the treatment of choice for all
these patients.
 NON-HODGKIN'S LYMPHOMA
(NHL)
• These are malignant tumours of the lymphoid system classified
separately from Hodgkin's lymphoma. Most (70%) are of B cell
origin although T cell tumours are increasingly being recognized.
• NHL is associated with the EBV virus (Burkitt's lymphoma) and the
human T cell lymphotropic virus which is prevalent in Japan, Africa,
South America and the Caribbean. Herpes virus 8 is associated with
primary effusion lymphomas and Castleman's disease; there is an
increase in lymphoma in patients with AIDS. Helicobacter pylori is
an aetiological factor in gastric lymphoma.
• Lymphomas also occur in congenital immunodeficiency, post-
transplantation and in autosomal family cancer syndromes .
• Other causes, e.g. occupation, dietary and exposure to chemicals,
have been linked to the increasing incidence but the evidence is
unconfirmed
 Treatment options
• Aggressive combination chemotherapy gives high complete
remission rates and molecular remission.
• Antibody therapy. The monoclonal antibody rituximab induces
remission (partial) in 30-70% of patients, almost without toxicity.
Molecular remissions are observed. Complications include the
cytokine release syndrome, with fever, vomiting and allergic
reactions (angio-oedema, bronchospasm and dyspnoea).
• Rituximab/chemotherapy combination. These have now been
reported to improve the complete remission rate (with
disappearance of Bcl-2 positive cells from the bone marrow in 100%
of patients), freedom from progression and event-free survival, even
though there is (as yet) no effect on overall survival. This may
become the standard therapy for CD20 positive lymphoma.
• Antibody-targeted irradiation.
 Clinical approach
• A careful history
, -Age of the patient.
-The occurrence of fever, sweats, or weight loss
– Site of infection, a particular medication, a travel history, or a previous malignancy.
• physical examination
– localized or generalized
– size of nodes
– Texture
– Mobility
– presence or absence of nodal tenderness
– signs of inflammation over the node
– skin lesions
– splenomegaly
• Imaging
– Chest radiography
– Ultrasonography
– Computed tomography
– Magnetic resonance imaging
– Positron emission tomography
• Sampling
– Needle aspiration
– Excisional biopsy
Neck lump
pleomorphic adenomaof
parroted gland
Submandibular duct stone
Multiple cervical metastases visible in the
nodal basins that drain the site of the
primary malignancy
Ludwigs angina
Ranula
Virchow`s lymph node
Branchial cyst
Thyroglossal cyst
Cystic Hygroma
 Neck lump
Thyroid, lymph node ,carotid ,salivary gland , sternomastoid muscle

• Goitre. Congenital
Neoplasm
1. • Thyroglossal duct cyst.
1. Thyroid neoplasms
2. • Cystic hygroma.
2. • Metastatic carcinoma.
3. • Primary lymphoma.
3. • Branchial cyst.
4. • Salivary gland tumour. 4. • Dermoid cyst.
5. • Sternocleidomastoid tumour. 5. • Torticollis.
6. • Carotid body tumour. Vascular
Inflammatory 1. .Caroted body tumors
1. • Acute infective adenopathy. 2. • Subclavian aneurysm.
2. • Collar stud abscess. 3. • Subclavian ectasia.
3. • Parotitis.,submandibular
sialadinitis
 Children
Congenital and inflammatory lesions are common.
• Cystic hygroma: in infants, base of the neck, brilliant transillumination.
• Thyroglossal or dermoid cyst: midline, discrete, elevates with
tongue protrusion.
• Torticollis: rock-hard mass, more prominent with head flexed,
associated with fixed rotation (a fibrous mass in the
sternocleidomastoid muscle).
• Branchial cyst: anterior to the upper third of the sternocleidomastoid.
• Viral/bacterial adenitis: usually affects jugular nodes, multiple,
tender masses.
• Neoplasms (lymphoma most common).
 Young adults
Inflammatory neck masses and thyroid
malignancy are common.
• Viral (e.g. infectious mononucleosis) or
bacterial (tonsillitis/pharyngitis) adenitis.
• Papillary thyroid cancer: isolated, non-
tender, thyroid mass, possible
lymphadenopathy.
 Older age group
Neck lumps are malignant until proven otherwise.
• Metastatic lymphadenopathy: multiple, hard, nontender,
tendency to be fixed.
• 75% in primary head and neck (thyroid, nasopharynx,
tonsils, larynx, pharynx), 25% from infraclavicular
primary (stomach, pancreas, lung).
• Primary lymphadenopathy (thyroid, lymphoma):
fleshy,rubbery, large size.
• Primary neoplasm (thyroid, salivary tumour): firm,
nontender, fixed to tissue of origin.