Lymph Node Disorders Treatment & Management

The medical therapy chosen is based on the most likely etiology if a biopsy has not been performed.

• In the case of bacterial infection, the most likely culprits include Staphylococcus and Streptococcus species; therefore, a beta-lactamase–resistant antibiotic is chosen. In patients with tuberculosis, rifampin and isoniazid are chosen.
• In cases of nontuberculous mycobacterial adenitis, most still advocate surgical management. However, some patients with lymphadenopathy in anatomic locations of concern may benefit from drugs such as clarithromycin, azithromycin, rifampin, rifabutin, or ethambutol.^[12]
• Most patients with viral etiology for lymphadenopathy may be treated expectantly.
• Patients with some of the more obscure diagnoses, such as Kawasaki disease, systemic lupus erythematosus (SLE), and Langerhans cells histiocytosis, may require immunosuppressants.

Enlargement of a cervical lymph node to 1 cm in diameter is considered abnormal and should be considered for biopsy if the diagnosis is otherwise uncertain. Biopsy of the lymph node may involve one of two methods. The most commonly used method is the surgical biopsy in which either a portion of the node or the complete node is excised.

Before the procedure, the patient and family are instructed in the steps involved and the risks and benefits; a consent form is obtained. Before removal of the node, the surgeon should discuss the case with the pathologist so the appropriate tests may be immediately performed after the specimen is received. The procedure is performed either in the operating room suite under general anesthetic or in a minor procedure room under conscious sedation.

An incision is made in the skin overlying the enlarged node, and the surrounding tissue is carefully dissected away from the node. Care must be taken to avoid surrounding nerves, especially in areas around the neck. To assist in the removal of the node, a suture on a noncutting needle may be placed through the center of the node to provide traction so it can be pulled into view (see the first and second images below). The also minimizes crush artifact that may result from excessive handling of the lymph node. The node must then be sent fresh to the pathologist for processing (see the third image below). This is to allow all possible tests to be performed, as fixation of the lymph node prevents the ability to perform some important tests (eg, flow cytology, cytogenetics). Usually, one large node or a group of smaller nodes is sent to the pathologist for diagnosis.

Although lymph node biopsy via an open technique is considered the standard approach, with the advent of minimally invasive techniques, surgeons are applying these methods to lymph node biopsies in the thoracic cavity and the abdomen. Although ultrasonography or CT-guided percutaneous lymph node biopsy often does not supply sufficient tissue for the histopathologic diagnosis of a lymphoma, laparoscopic lymph node biopsy has the advantage of obtaining the entire lymph node and avoiding the invasiveness and possible complications of a laparotomy.^[13]

Lymph node biopsies are usually performed on an outpatient basis. Before the patient is discharged from the hospital, the wound is assessed for swelling and bleeding. The wound area should be kept dry for at least 2 days, and appropriate analgesia should be administered.

Patients and their families should be contacted with the results as soon as the report is finalized. If further therapy is necessary, patients should return to the hospital or be referred to the appropriate specialists for therapy.

The known complications of the biopsy itself arise from the injury of surrounding structures around the node, including the soft tissue, blood vessels, and nerves. Other potential complications in patients with malignancy include the spread of tumor cells in the area of the biopsy, production of a draining sinus in the case of atypical Mycobacterium infection (if the entire node is not excised), and the risks associated with general anesthetics, especially if the patient has an anterior mediastinal mass.

Viral-associated lymphadenopathy

Most commonly, upper respiratory tract infections predominate as the source of lymphadenopathy. The nodes tend to be small, soft, and bilateral and do not have warmth or erythema of the overlying skin. Cervical adenopathy is a prominent feature of EBV and CMV. Posterior cervical nodes are most commonly involved, followed by the anterior cervical chain. Children with adenoviral-associated respiratory infections may present with generalized constitutional symptoms and bilateral cervical adenopathy. Treatment is based on controlling symptoms and preventing complications instead of providing specific antiviral therapies.^[2]

Bacterial-associated lymphadenopathy

The two most common organisms associated with lymphadenopathy include S aureus and group B streptococci. The clinical history often reveals a recent sore throat or cough, whereas the physical examination findings include impetigo, pharyngitis, tonsillitis, or acute otitis media. The primary sites involved include the submandibular, upper cervical, submental, occipital, and lower cervical nodal regions. The treatment involves administration of beta-lactamase–resistant antibiotics and drainage of purulence when fluctuation is present.^[14]

With respect to children with acute adenitis, children hospitalized for a first episode of acute unilateral infectious adenitis generally do well. Younger patients and those with longer duration of node involvement before admission have an increased risk of surgical node drainage.^[15]

Atypical mycobacterium

In the United States, atypical Mycobacterium account for most cases of adenitis due to Mycobacterium infection. Numerous members are in this group, including Mycobacterium scrofulaceum and Mycobacterium avium-intracellulare. The onset of adenopathy may be relatively sudden; size may gradually increase over 2-3 weeks. The involved nodes usually have an overlying erythema and may be tender. The nodes may progress to fluctuance and ultimately spontaneously drain. Treatment involves complete excision of the involved node because incision and drainage may lead to a chronically draining sinus.^[16] Those dissections that may be adjacent to the marginal mandibular nerve are often associated with a transient paralysis that resolves over a few months.^[17]

Mycobacterium tuberculosis

Lymph node involvement with Mycobacterium tuberculosis is commonly referred to as scrofula. It was previously a well-known manifestation of extrapulmonary tuberculosis; however, as tuberculosis has declined, so has the incidence of scrofula. Nonetheless, it is still prevalent in much of the world; patients with scrofula present with cervical node enlargement, most often around the paratracheal nodes or the supraclavicular nodes. Abnormal findings are observed on chest radiography in most cases. Clinical features are not helpful in distinguishing atypical from tuberculous mycobacterial infections. Nodal enlargement is usually painless; nodes are likely to suppurate and form sinuses. Performing a tuberculin test is usually helpful. Treatment involves administration of rifampin and isoniazid.^[18]

Catscratch disease

Catscratch disease is a zoonotic infection that originates from animal scratches, most likely cat or kitten scratches. The primary inoculation of the skin, eye, or mucosal membrane leaves a small papule that may or may not be evident upon examination. Indeed, the papule may resolve before the lymphadenitis appears. Patients usually have accompanying constitutional symptoms, such as fever, malaise, and fatigue. The causative agent is Bartonella henselae, a gram-negative rickettsial organism. The disease is usually self-limiting and requires only supportive treatment.

Malignancies

Patients with lymphoma (Hodgkin disease [HD], non-Hodgkin lymphoma [NHL]), leukemia, or metastatic solid tumors may present with lymphadenopathy. The nodes are usually painless and continue 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, a biopsy is needed to establish the diagnosis and allow important tests to be performed to guide therapy.

In a retrospective review by Oguz et al, children referred for concerning lymphadenopathy were more likely to have a malignant etiology if the lymph node was larger than 3 cm in size, the enlargement lasted longer than 4 weeks, supraclavicular involvement was observed, and abnormal laboratory and radiological findings were noted.^[19]

Other causes of adenopathy

Many less common disorders may also appear as lymphadenopathy.

In Kawasaki disease (ie, mucocutaneous lymph node syndrome), lymphadenitis is one of the earliest aspects of the disease. The enlarged node or group of nodes are unilateral, nonfluctuant, and usually located in the anterior triangle of the neck. Resolution of lymphadenitis is a rule.

Enlarged lymph nodes are prominent features in the course of sarcoidosis; the supraclavicular nodes and bilateral hilar nodes are involved.

Kikuchi lymphadenitis (ie, histiocytic necrotizing lymphadenitis) is a benign and rare disease of unknown origin that involves bilaterally enlarged cervical lymph nodes that are unresponsive to antibiotic therapy. Patients with Kikuchi lymphadenitis often have systemic symptoms, including fever, hepatosplenomegaly, and weight loss.

SLE often involves enlarged lymph nodes. Children with SLE tend to have more organ systems involved and a more severe course than adults with SLE.

Langerhans cell histiocytosis (ie, histiocytosis X) is a syndrome with a broad clinical spectrum; its unifying pathologic feature is the derivation from Langerhans cells. The disease is believed to be a clonal neoplasm in which lymph node enlargement is common.

Lymph node enlargement is a common feature of various benign and malignant disorders that affect children. If the history and physical examination are thorough, the etiology of most lymphadenopathies can be determined without further investigation. However, if the diagnosis needs to be confirmed or is in doubt, the results from a carefully chosen combination of skin tests, serologic tests, and/or diagnostic imaging tests may establish the correct diagnosis. If the diagnosis is still unclear or if tissue is required in the case of a potential malignancy, the results from a careful lymph node biopsy can most certainly confirm the correct diagnosis.

Lymphadenopathy is present in a vast array of disorders, and discussing the future of lymphadenopathy is difficult because of the number of diseases involved. The diagnosis of lymph node disorders will improve as molecular tools become more available. Having these tools will allow clinicians to diagnose the etiology with more exact science and less invasive means. The use of FNA in children will become more frequent as more experience is obtained in centers that are currently not using this technique.

For patient education resources, see the Blood and Lymphatic System Center, as well as Swollen Lymph Glands.