Conditions of the Lymph System

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Conditions of the Lymph System
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Anatomy of head neck lymph nodes
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Anatomy of axillary lymph nodes
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Anatomy of inguinal lymph nodes
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• Generalized adenopathy has been defined as
• involvement of three or more
  noncontiguous lymph node areas.

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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)

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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

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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

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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)

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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

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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

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syphilis

• Clinical Symptoms may evolve
• Generalized painless lymphadenopathy
• Maculo-papular, papular, or pustular rash on
  entire body, especially on palms and soles
• Highly infectious lesions on mucous membranes
  (lips, mouth, pharynx, vulva, glans penis) which
  are silvery grey superficial erosions with a red
  halo and not painful unless there is a secondary
  infection.
• 40 of these patients will have CNS involvement
  with headache and meningismus
• 1-2 will develop acute aseptic meningitis
• benzathine penicillin 2.4 million units IM single
  dose

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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.

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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 (

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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 gt 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 (

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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

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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 gt 38C), 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 single extranodal site
  that is contiguous or proximal to the known nodal
  site

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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.

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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

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Types

• Follicular
• Lymphoplasmacytict
• Mantle cell
• Diffuse large B cell
• Burkitts
• Anaplastic
• MALT (mucosal associated lymphoid tissue)

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WHO classification of lymphoid neoplasms

• B cell lymphomas
• Precursor B cell lymphoma Precursor B
  lymphoblastic lymphoma/leukaemia (highly
  (aggressive)
• Mature B cell lymphoma
• Chronic lymphocytic leukaemia/small
  lymphocytic lymphomaLymphoplasmacytic
  lymphomaSplenic marginal zone lymphomaExtranodal
  marginal zone B cell lymphoma of
  mucosa-associated lymphoid tissue
  (MALT-lymphom)Nodal marginal zone B cell
  lymphomaFollicular lymphoma((aggressive)Mantle
  cell lymphomaDiffuse large B cell lymphoma)
  aggressiv)Mediastinal (thymic) large B cell
  lymphomaIntravascular large B cell
  lymphomaPrimary effusion lymphomaBurkitt's
  lymphoma/leukaemia (highly aggressive)
• T/NK cell lymphomas
• Precursor T cell lymphoma
• Precursor T cell lymphoblastic
  leukaemia/lymphoma(highly aggressive)
• Blastic NK cell lymphoma
• Mature T/NK cell lymphoma
• Adult T cell leukaemia/lymphoma(very
  aggressive(Extranodal NK/T cell lymphoma, nasal
  typeEnteropathy-type T cell lymphomaHepatospleni
  c T cell lymphomaSubcutaneous panniculitis-like
  T cell lymphomaMycosis fungoidesSézary
  syndromePrimary cutaneous anaplastic large cell
  lymphomaPeripheral T cell lymphoma, unspecified
  (aggressive)Angioimmunoblastic T cell
  lymphomaAnaplastic large cell lymphoma
  (aggressive)

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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.

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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

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The Lymphatics
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Lymphedema

• Lymphedema is the result of an inability of the
  existing lymphatic system to accommodate the
  protein and fluid entering the interstitial
  compartment at the tissue level. In the first
  stage of lymphedema, impaired lymphatic drainage
  results in protein-rich fluid accumulation in the
  interstitial compartment. Clinically, this
  manifests as soft pitting edema.
• In the second stage of lymphedema, the clinical
  condition is further exacerbated by accumulation
  of fibroblasts, adipocytes, and, perhaps most
  importantly, macrophages in the affected tissues,
  which culminate in a local inflammatory response.
  This results in important structural changes from
  the deposition of connective tissue and adipose
  elements at the skin and subcutaneous level. In
  the second stage of lymphedema, tissue edema is
  more pronounced, is nonpitting, and has a spongy
  consistency.
• In the third and most advanced stage of
  lymphedema, the affected tissues sustain further
  injury as a result of both the local inflammatory
  response as well as recurrent infectious episodes
  that typically result from minimal subclinical
  skin breaks in the skin. Such repeated episodes
  injure the incompetent, remaining lymphatic
  channels, progressively worsening the underlying
  insufficiency of the lymphatic system. This
  eventually results in excessive subcutaneous
  fibrosis and scarring with associated severe skin
  changes characteristic of lymphostatic
  elephantiasis

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Clinical picture

• The edematous limb has a firm and hardened
  consistency.
• There is loss of the normal perimalleolar shape,
  resulting in a tree trunk pattern.
• The dorsum of the foot is characteristically
  swollen, resulting in the appearance of the
  buffalo hump, and the toes become thick and
  squared .
• In advanced lymphedema, the skin undergoes
  characteristic changes, such as lichenification,
  development of peau dorange, and
  hyperkeratosis.Additionally, the patients give a
  history of recurrent episodes of cellulitis and
  lymphangitis after trivial trauma and frequently
  present with fungal infections affecting the
  forefoot and toes.
• Patients with isolated lymphedema usually do not
  have the hyperpigmentation or ulceration one
  typically sees in patients with chronic venous
  insufficiency.
• Lymphedema does not respond significantly to
  overnight elevation, whereas edema secondary to
  central organ failure or venous insufficiency
  does.

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Differential Diagnosis

• The most common causes of bilateral extremity
  edema are of systemic origin. The most common
  etiology is cardiac failure, followed by renal
  failure. Hypoproteinemia secondary to cirrhosis,
  nephrotic syndrome, and malnutrition can also
  produce bilateral lower extremity edema.
• Another important cause to consider with
  bilateral leg enlargement is lipedema. Lipedema
  is not true edema but rather excessive
  subcutaneous fat found in obese women. It is
  bilateral, nonpitting, and greatest at the ankle
  and legs, with characteristic sparing of the
  feet. There are no skin changes,and the
  enlargement is not affected by elevation. The
  history usually indicates that this has been a
  lifelong problem that runs in the family.
• Once the systemic causes of edema are excluded,
  in the patient with unilateral extremity
  involvement, edema secondary to venous and
  lymphatic pathology should be entertained. The
  edema responds promptly to overnight leg
  elevation. In the later stages, the skin is
  atrophic with brawny pigmentation. Ulceration
  associated with venous insufficiency occurs above
  or posterior and beneath the malleoli.

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CLASSIFICATION of lymph- edema

• Lymphedema is generally classified as primary
  when there is no known etiology and secondary
  when its cause is a known disease or disorder.
• Primary lymphedema has generally been classified
  on the basis of the age at onset and presence of
  familial clustering.
• Primary lymphedema with onset before the first
  year of life is called congenital. The familial
  version of congenital lymphedema is known as
  Milroys disease and is inherited as a dominant
  trait.
• Primary lymphedema with onset between the ages of
  1 and 35 years is called lymphedema praecox. The
  familial version of lymphedema praecox is known
  as Meiges disease.
• Finally, primary lymphedema with onset after the
  age of 35 is called lymphedema tarda.
• Worldwide the most common cause of secondary
  lymphedema is infestation of the lymph nodes by
  the parasite Wuchereria bancrofti in the disease
  state called filariasis. In the developed
  countries the most common causes of secondary
  lymphedema involve resection or ablation of
  regional lymph nodes by surgery, radiation
  therapy, tumor invasion, direct trauma, or, less
  commonly, an infectious process.

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Investigation

• The diagnosis of lymphedema is relatively easy in
  the patient who presents in the second and third
  stages of the disease. It can however, be a
  difficult diagnosis to make in the first stage,
  particularly when the edema is mild, pitting, and
  relieved with simple maneuvers such as elevation.
  For patients with suspected secondary forms of
  lymphedema, computed tomography (CT) and magnetic
  resonance imaging (MRI) are valuable and indeed
  essential for exclusion of underlying oncologic
  disease
• In patients with known lymph node excision and
  radiation treatment as the underlying problem of
  their lymphedema, additional diagnostic studies
  are rarely needed except as these studies relate
  to follow-up of an underlying malignancy.
• For patients with edema of unknown etiology and a
  suspicion for lymphedema, lymphoscintigraphy is
  the diagnostic test of choice.
• When lymphoscintigraphy confirms that lymphatic
  drainage is delayed, the diagnosis of primary
  lymphedema should never be made until neoplasia
  involving the regional and central lymphatic
  drainage of the limb has been excluded through CT
  or MRI.
• If a more detailed diagnostic interpretation of
  lymphatic channels is needed for operative
  planning, then contrast lymphangiography may be
  considered.

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THERAPY

• The large majority of lymphedema patients can be
  treated with a combination of limb elevation, a
  high-quality compression garment, complex
  decongestive physical therapy, and compression
  pump therapy.
• Operative treatment may be considered for
  patients with advanced complicated lymphedema
  that fail management with nonoperative means.

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Neck lump
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pleomorphic adenomaof parroted gland
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Multiple cervical metastases visible in the nodal
basins that drain the site of the primary
malignancy
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Ludwigs angina
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Ranula
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Virchows lymph node
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Branchial cyst
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Thyroglossal cyst
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Cystic Hygroma
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Neck lumpThyroid, lymph node ,carotid ,salivary
gland , sternomastoid muscle

• Goitre.
• Neoplasm
• Thyroid neoplasms
• Metastatic carcinoma.
• Primary lymphoma.
• Salivary gland tumour.
• Sternocleidomastoid tumour.
• Carotid body tumour.
• Inflammatory
• Acute infective adenopathy.
• Collar stud abscess.
• Parotitis.,submandibular sialadinitis

• Congenital
• Thyroglossal duct cyst.
• Cystic hygroma.
• Branchial cyst.
• Dermoid cyst.
• Torticollis.
• Vascular
• .Caroted body tumors
• Subclavian aneurysm.
• Subclavian ectasia.

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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).

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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.

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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.