Disorders of Lymphatic System

7/29/2019 Disorders of Lymphatic System

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Disorders of lymphatic system

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Lymphadenopathy

Lymphadenopathy is the enlargement of

one or more lymph nodes as a result of

normal reactive effects or a pathologic

occurrence.

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Etiology:

An immune response to infective agents (eg, bacteria,virus, fungus)

Inflammatory cells in infections involving the lymphnode

Infiltration ofneoplastic cells carried to the node bylymphatic or blood circulation (metastasis)

Localized neoplastic proliferation of lymphocytes ormacrophages (eg, leukemia, lymphoma)

Infiltration of macrophages filled with metabolitedeposits (eg, storage disorders)

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A localized lymphadenopathy usually resultsfrom abnormalities of the area in which thelymph node drains, although it cannot beexcluded as the first sign of a precociousclinical manifestation in the course of aprogressive systemic process

The appearance of a generalizedlymphadenopathy orients the clinician moredirectly toward serologic and hematologictesting.

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Regional lymphadenopathies

Of the regional lymphadenopathies, occipital

and preauricular locations are rarely

malignant;

The occipital are often related to scalp andouter ear infections, exanthematous diseases,

and toxoplasmosis,

The preauricularare associated with

infections of superficial tissue of the orbit, the

middle ear, and the parotid glands.

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Submental lymphadenopathy requires a

search for disorders in the anterior portion of

the mouth and the lower lip, submental

salivary gland. The submandibularlymphadenopathy with

disorders in submandibular portion of the face,

the nose, the maxillary sinus, the mucosa of

the oral cavity, the floor of the mouth, and thesubmandibular salivary gland

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Laterocervical lymphadenopathy in theupper portion of the neck can beassociated with inflammatory or

neoplastic disorders of the hypopharynx,the larynx, or the thyroid gland,

Those in the lowerpart of the neck arerelated to disorders of the hypoglotticlarynx, the thyroid, and the upper portionof the esophagus.

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Supraclavicular and epitrochlear

enlargement must be considered as

red flagsfor the potential of malignancy.

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Axillary lymphadenopathy is seen with

infections of the upper extremity, chest wall,

breast tissue, and intrathoracic lesions.

Inguinal lymphadenopathies are caused bysexually transmitted diseases of the genitalia

and other infections of the perineum and

pelvis.

Enlarged popliteal lymph nodes are generally

associated with infections of the foot and leg.

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Lymphadenopathies of the mediastinum,

retroperitoneum, and mesentery are

usually not detected at the time of

physical examination but are sometimessuspected by compression of the

surrounding structures.

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General lymphadenopathy

Any of the common viral illnesses may producegeneralized lymphadenopathy (eg, Epstein-Barr virus[EBV], cytomegalovirus [CMV], humanimmunodeficiency virus [HIV]).

Hematogenous malignancies (eg, leukemia,lymphomas)

Oethrmalignancies (eg, neuroblastoma,rhabdomyosarcoma).

Some rare causes of generalized lymphadenopathyinclude autoimmune connective tissue diseases anduse of certain drugs, particularly phenytoin andcarbamazepine.

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In most instances, lymph nodes up to 1

cm can still be considered normal. The

two exceptions to this rule include the

epitrochlear node in which up to 0.5 cmis allowed and the inguinal nodes in

which up to 1.5 cm is allowed.

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A complete blood cell count with a manualdifferentiation provides useful information.

leukemias are often accompanied by pancytopenia.

A predominantly lymphocytic elevation (>1 X 109

cells/L) is practically diagnostic ofmononucleosis; when the proportion of these cells is less elevated but

still predominant, CMV and toxoplasmosis must beconsidered. Finding medium-to-large lymphocytes thatcan be classified as in transformation or activated isuseful to indicate a viral infection.

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Serum lactate dehydrogenase (LDH)may be used to determine the turnoverrate of cells in the case of leukemia or

lymphoma. Other tests, such as tuberculin skin test;

monospot; and titers for EBV, CMV,catscratch disease, or toxoplasmosis,may be performed to evaluate forspecific etiologies

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Imaging Studies

Chest radiography may be useful to assess

for potential sources of infection, such as

bacterial pneumonias or tuberculosis, and hilar

adenopathy in the case of malignancy. Indeed, because numerous reports describe

airway collapse with anesthetics in the case of

a large anterior mediastinal mass, chest

radiography should be considered before anygeneral anesthetic is administered.

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Ultrasonography may be performed to distinguish thelymph nodal nature if palpation is not sufficient.Furthermore, it may be used to distinguish theabnormality from other potential anatomicstructures (eg, dermoid cysts, thyroglossal duct cysts,

branchial cleft cysts, inguinal hernias, undescendedtesticles).

Ultrasonography may reveal relationships tocontiguous structures

Offer information about the content of the enlarged

lymph node or nodes (ie, solid, liquid, gas,homogeneous or nonhomogenous).

Finally, ultrasonography has been used in an effort toestablish etiology based on ultrasonographiccharacteristics

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(CT) scanning is useful to depict deep lymphnodes, especially in the thoracic andabdominal cavities. This may be the onlynoninvasive technique available to evaluatethese areas and determine a potential sourceofmalignancy (eg, neuroblastoma, Burkittlymphoma, rhabdomyosarcoma).

Furthermore, chest CT scanning depict ananterior mediastinal mass as well as the extentof tracheal or bronchial airway compression

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18F-fluorodeoxyglucose positron

emission tomography (18F-FDG PET)

has been used in adult patients with

lymphoma and more recently in childrento assist in diagnosis and to monitor

disease during therapy with promising

findings.

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Diagnostic Procedures

(FNA) biopsy has been used extensively inadults and is being described in children

The cited advantages of FNA biopsy are thatit can be performed in the outpatient

department, is simple and rapid, does notrequire a general anesthetic, has lowmorbidity, is cost effective, and producesminimal scarring

The sensitivity and specificity of FNA biopsy indetermining the etiology of lymphadenopathyare more than 90%

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The limits include center dependence onpathologists, the potential risk ofseeding atract with malignancy, and the continued needfor at least conscious sedation in mostchildren. Most oncology protocols now requirespecial studies to be performed on the nodaltissue, including cytogenetics, flow cytometry,electron microscopy, and special stains that

FNA does not allow. To obtain more tissue, some investigators

have used core needle techniques

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Excisional biopsy

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Contraindications:

An absolutecontraindication to lymph node biopsy

exists if the etiology is clearand the lymphadenopathy

is expected to improve with no further management.

A relat ivecontraindication exists if the suspected

etiology can be treated expectantly, eg, in cases of

bacterial infection of the node in which the use of

antibiotics is expected to improve the clinical scenario.

Another relative contraindication exists if an anterior

mediastinal mass is noted on chest radiography andconsidered to be a high anesthetic risk.

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The complications

The injury of surrounding structures aroundthe nodethe soft tissue, blood vessels, andnerves.

With malignancy the spread of tumor cells in

the area of the biopsy, Production of a draining sinus in the case of

atypical Mycobacterium if the entire node isnot excised,

The risks associated with generalanesthetics, especially if the patient has ananterior mediastinal mass.

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TB

In the United States, atypical

Mycobacterium account for most cases

of adenitis due to Mycobacterium

infection.

Present with cervical node enlargement,

most often around the paratracheal

nodes or the supraclavicular nodes.

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Nodal enlargement is usually painless; nodes

are likely to suppurate and form sinuses.

Clinical features are not helpful in

distinguishing atypical from tuberculousmycobacterial infections.

Performing a tuberculin test is usually helpful.

Abnormal findings are observed on chestradiography in most cases.

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Treatment involves administration of

rifampin and isoniazid

Treatment involves complete excision of

the involved node because incision and

drainage may lead to a chronically

draining sinus

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Malignancies

The nodes are usually painless andcontinue to enlarge.

Inflammatory signs or focuses are

usually absent.Associated B symptoms of HD may be

present, including fever, night sweats,

weight loss, and malaise. If malignancy is suspected, biopsy is

needed to establish the diagnosis

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The diagnosis of lymph node disorders

will improve as molecular tools become

more available,which will allow clinicians

to diagnose the etiology with more exactscience and less invasive means.

The use of FNA in children will become

more frequent

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Lymphedema

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Lymphedema is a notoriously debilitating

progressive condition with no known

cure.

The underlying problem is lymphatic

dysfunction, resulting in an abnormal

accumulation of interstitial fluid

containing high molecular weightproteins.

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Primarylymphedema represents a

developmental abnormality of the lymphatic

system.

Primary lymphedema has been further subdividedinto 3 forms, including congenital lymphedema,

lymphedema praecox, and lymphedema tarda,

depending on age at presentation.

These conditions are most often sporadic, with nofamily history, and involve the lower extremity

almost exclusively.

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In the United States, the highest incidence oflymphedema is observed following breast cancersurgery, particularly among those who undergoradiation therapy following axillary lymphadenectomy.

Among this population, 10-40% develop some degreeof ipsilateral upper extremity lymphedema.

Worldwide, 140-250 million cases of lymphedema areestimated to exist, with filariasis being the mostcommon cause.

o Other causes include vein stripping, peripheral vascularsurgery, lipectomy, burns, burn scar excision, and insect bites.

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Clinically

Patients present with varying degrees of severity, frommild swelling to severe disabling enlargement withpotentially life-threatening complications.

Asymmetry or increased circumference of an

extremity. Difficulty fitting into clothing.

may cause fatigue related to the size and weight ofthe extremity, and severe impairment of dailyactivities.

Recurrent bacterial or fungal infections are alsocommon

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Patients with chronic lymphedema for10 years have a10% risk of developing lymphangiosarcoma,

commonly present with a reddish purple discolorationor nodule that tends to form satellite lesions. It may

be confused with Kaposi sarcoma or traumaticecchymosis.

The 5-year survival rate is less than 10%, and averagesurvival following diagnosis is 19 months.

This malignant degeneration is most commonlyobserved in patients with postmastectomylymphedema (Stewart-Treves syndrome), whereincidence is estimated to be 0.5%.

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D.D.

Other causes of edema, such as edemasecondary to congestive heart failure, renalinsufficiency, hepatic insufficiency, or venousstasis disease, must be excluded.

Malignancymust always be considered,particularly when patients report sudden onset,rapid progression, or associated pain. Thesesymptoms may indicate direct tumor growth or

metastatic disease in the regional lymph nodebasin.

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Lymphoscintigraphy has replacedlymphangiography

An indication for CT scan orMRI is suspicionof malignancy,

Dopplerultrasonography is also used bysome to evaluate flow in the lymphatic andvenous systems.

The presence of a deep vein thrombosis is in thedifferential diagnosis of unilateral extremityswelling, and it may also occur concomitantly withlymphedema.

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Surgical treatment is palliative, not curative,and it does not obviate the need for continuedmedical therapy.

Moreover, it is rarely indicated as the primarytreatment modality. Rather, reserve surgicaltreatment for those who do not improve withconservative measures or in cases where theextremity is so large that it impairs daily

activities and prevents successful conservativemanagement.

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Physiologic procedures attempt toimprove lymphatic drainage.

omental transposition,

enteromesenteric bridging, buried dermal flaps,

lymphangioplasty,

microvascular lympholymphatic orlymphovenous anastomoses.

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Excisional techniques remove the affected

tissues, thus reducing the lymphedema load.

The Charles procedure is quite radical

excisional technique. This procedure involvesthe total excision of all skin and subcutaneous

tissue from the affected extremity. The

underlying fascia is then grafted, using the

skin that has been excised

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Staged excision has become the option ofchoice for many authors and is described ingreater detail.

This procedure involves removing only a

portion of skin and subcutaneous tissue,followed by primary closure.

After approximately 3 months, the procedure isrepeated on a different area of the extremity.This procedure is safe, reliable, anddemonstrates the most consistentimprovement with the lowest incidence ofcomplications.

Disorders of Lymphatic System

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