Lymphadenopathy

Lymphadenopathy
Synonyms	Adenopathy
Neck lymphadenopathy associated with infectious mononucleosis
Classification and external resources
Specialty	Infectious disease
ICD-10	I88, L04, R59.1
ICD-9-CM	289.1-289.3, 683, 785.6
DiseasesDB	22225
MedlinePlus	001301
eMedicine	ped/1333
MeSH	D008206

Lymphadenopathy or adenopathy is disease of the lymph nodes, in which they are abnormal in size, number, or consistency.^[1] Lymphadenopathy of an inflammatory type (the most common type) is lymphadenitis,^[2] producing swollen or enlarged lymph nodes. In clinical practice, the distinction between lymphadenopathy and lymphadenitis is rarely made and the words are usually treated as synonymous. Inflammation of the lymphatic vessels is known as lymphangitis.^[3] Infectious lymphadenitides affecting lymph nodes in the neck are often called scrofula.

The term comes from the word lymph and a combination of the Greek words αδένας, adenas ("gland") and παθεία, patheia ("act of suffering" or "disease").

Lymphadenopathy is a common and nonspecific sign. Common causes include infections (from minor ones such as the common cold to dangerous ones such as HIV/AIDS), autoimmune diseases, and cancers. Lymphadenopathy is also frequently idiopathic and self-limiting.

Classification

Micrograph of dermatopathic lymphadenopathy, a type of lymphadenopathy. H&E stain.

CT scan of axillary lymphadenopathy in a 57 year old man with multiple myeloma.

• By size, lymphadenopathy in adults is present when the short axis of one or more lymph nodes is greater than 10mm.^[4][5] However, there is regional variation as detailed in this table:

Lymphadenopathy of the axillary lymph nodes can be defined as solid nodes measuring more than 1.5 cm without fatty hilum.^[11] Axillary lymph nodes may be normal up to 3 cm if consisting largely of fat.^[11]

In children, a short axis of 8 mm can be used.^[12] However, inguinal lymph nodes of up to 1.5 and cervical lymph nodes of up to 2 cm are generally normal in children up to age 8–12.^[13]

• By extent:
  • Localized lymphadenopathy: due to localized spot of infection e.g., an infected spot on the scalp will cause lymph nodes in the neck on that same side to swell up
  • Generalized lymphadenopathy: due to a systemic infection of the body e.g., influenza or secondary syphilis
    • Persistent generalized lymphadenopathy (PGL): persisting for a long time, possibly without an apparent cause
• By malignancy: Benign lymphadenopathy is distinguished from malignant causes which mainly refer to lymphomas or lymph node metastasis
• By localization, including hilar lymphadenopathy.
• Dermatopathic lymphadenopathy: lymphadenopathy associated with skin disease.

Causes

Retroperitoneal lymphadenopathies of testicular seminoma, embrace the aorta. Computed tomography image.

Lymph node enlargement is recognized as a common sign of infectious, autoimmune, or malignant disease. Examples may include:

• Reactive: acute infection (e.g., bacterial, or viral), or chronic infections (tuberculous lymphadenitis,^[14] cat-scratch disease^[15]).
  • The most distinctive sign of bubonic plague is extreme swelling of one or more lymph nodes that bulge out of the skin as "buboes." The buboes often become necrotic and may even rupture.^[16]
  • Infectious mononucleosis is an acute viral infection caused by Epstein-Barr virus and may be characterized by a marked enlargement of the cervical lymph nodes.^[17]
  • It is also a sign of cutaneous anthrax^[18] and Human African trypanosomiasis^[19]
  • Toxoplasmosis, a parasitic disease, gives a generalized lymphadenopathy (Piringer-Kuchinka lymphadenopathy).^[20]
  • Plasma cell variant of Castleman's disease - associated with HHV-8 infection and HIV infection^[21][22]
  • Mesenteric lymphadenitis after viral systemic infection (particularly in the GALT in the appendix) can commonly present like appendicitis.^[23][24]

Less common infectious causes of lymphadenopathy may include bacterial infections such as cat scratch disease, tularemia, brucellosis, or prevotella.

• Tumoral:
  • Primary: Hodgkin lymphoma^[25] and non-Hodgkin lymphoma give lymphadenopathy in all or a few lymph nodes.^[20]
  • Secondary: metastasis, Virchow's Node, neuroblastoma,^[26] and chronic lymphocytic leukemia.^[27]
• Autoimmune: systemic lupus erythematosus^[28] and rheumatoid arthritis may have a generalized lymphadenopathy.^[20]
• Immunocompromised: AIDS. Generalized lymphadenopathy is an early sign of infection with human immunodeficiency virus (HIV), the virus that causes acquired immunodeficiency syndrome (AIDS).^[29] "Lymphadenopathy syndrome" has been used to describe the first symptomatic stage of HIV progression, preceding a diagnosis of AIDS.
• Bites from certain venomous snakes such as the pit viper^[30]
• Unknown: Kikuchi disease,^[31] progressive transformation of germinal centers, sarcoidosis, hyaline-vascular variant of Castleman's disease, Rosai-Dorfman disease,^[32] Kawasaki disease,^[33] Kimura disease^[34]

Benign (reactive) lymphadenopathy

Benign lymphadenopathy is a common biopsy finding, and may often be confused with malignant lymphoma. It may be separated into major morphologic patterns, each with its own differential diagnosis with certain types of lymphoma. Most cases of reactive follicular hyperplasia are easy to diagnose, but some cases may be confused with follicular lymphoma. There are seven distinct patterns of benign lymphadenopathy:^[17]

• Follicular hyperplasia: This is the most common type of reactive lymphadenopathy.^[17]
• Paracortical hyperplasia/Interfollicular hyperplasia: It is seen in viral infections, skin diseases, and nonspecific reactions.
• Sinus histiocytosis: It is seen in lymph nodes draining limbs, inflammatory lesions, and malignancies.
• Nodal extensive necrosis
• Nodal granulomatous inflammation
• Nodal extensive fibrosis (Connective tissue framework)
• Nodal deposition of interstitial substance

These morphological patterns are never pure. Thus, reactive follicular hyperplasia can have a component of paracortical hyperplasia. However, this distinction is important for the differential diagnosis of the cause.

Localization

• Mediastinal lymphadenopathy
• Bilateral hilar lymphadenopathy

See also

• Adenitis
• Lymphovascular invasion

References

External links

• HPC:13820 on humpath.com (Digital slides)