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Conditions of the Lymph System

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Lymphedema does not respond significantly to overnight elevation, whereas edema secondary to central organ failure or venous insufficiency does. – PowerPoint PPT presentation

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Title: Conditions of the Lymph System


1
Conditions of the Lymph System
2
Anatomy of head neck lymph nodes
3
Anatomy of axillary lymph nodes
4
Anatomy of inguinal lymph nodes
5
  • Generalized adenopathy has been defined as
  • involvement of three or more
    noncontiguous lymph node areas.

6
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)

7
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

8
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

9
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)

10
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

11
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

12
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

13
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.

14
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15
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 (

16
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 (

17
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

18
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

19
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.

20
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

21
Types
  • Follicular
  • Lymphoplasmacytict
  • Mantle cell
  • Diffuse large B cell
  • Burkitts
  • Anaplastic
  • MALT (mucosal associated lymphoid tissue)

22
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)

23
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.

24
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

25
The Lymphatics
26
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

27
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.

28
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.

29
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.

30
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.

31
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.

32
Neck lump
33
pleomorphic adenomaof parroted gland
34
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35
Multiple cervical metastases visible in the nodal
basins that drain the site of the primary
malignancy
36
Ludwigs angina
37
Ranula
38
Virchows lymph node
39
Branchial cyst
40
Thyroglossal cyst
41
Cystic Hygroma
42
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43
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44
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.

45
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).

46
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.

47
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.
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