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Lymphadenopathy 476 Archives ofDisease in Childhood 1995; 73: 476-479 PERSONAL PRACTICE Arch Dis Child: first published as 10.1136/adc.73.5.476 on 1 November 1995. Downloaded from Lymphadenopathy Bruce Morland Lymph node enlargement is a common finding < 1 cm diameter are invariably of non-specific on routine physical examination of children. aetiology,' others agree with '1 cm rule', but The dilemma facing general practitioners is add that epitrochlear nodes >05 cm, and when to refer to paediatricians; the dilemma inguinal nodes > 1-5 cm should be regarded as for paediatricians is deciding how extensively abnormal nodal dimensions in these anatom- to investigate children. While the majority of ical sites.2 3 Full examination of all nodal cases will have a benign, rapidly resolving sites should be undertaken to determine iflym- course, the well recognised associations of phadenopathy is localised or generalised. The lymphadenopathy and potentially life threaten- distinction between localised or generalised ing diseases such as cancer cause worry and lymphadenopathy is important, as a specific anxiety to both families and doctors caring for pathological cause is more likely to be found in children. This concern may in itself initiate patients with generalised lymphadenopathy.I an inappropriately rapid or over aggressive The descriptive character ofthe lymph node(s) attempt at determining the diagnosis. An (firm, rubbery, fixed), is at best subjective and organised approach to children with lym- at worst quite misleading. Far from being phadenopathy will help the clinician make pathognomonic of malignant nodes, many appropriate decisions regarding treatment and inflammatory lesions especially if associated further investigation. with fibrotic reaction can 'feel sinister'. Abdominal palpation should determine the presence of liver or splenic enlargement. History Features of anaemia, petechiae, or bleeding A detailed history should focus both on diag- will point.towards a marrow infiltrative disease nostic clues, and to features suggestive of more such as leukaemia. The skin should be exam- sinister pathology. Duration of lymphadeno- ined for infective lesions or exanthematous pathy may be helpful, most infectious causes rashes. A careful ear, nose, and throat exami- http://adc.bmj.com/ producing a short (less than two week) history. nation, especially in children with cervical Long standing lymphadenopathy may be lymphadenopathy, with particular emphasis on caused by a variety of diseases including infec- nasal discharge, obstruction, or depression of tions (HIV, Epstein-Barr virus, tuberculosis, the soft palate helps to define possible source etc), malignancy, inflammation, and auto- of infection while considering some of the immune disease, but while very long standing commoner malignant head and neck tumours (over one year) lymph node enlargement is such as non-Hodgkin's lymphoma, rhabdo- likely to be pathological, it is unlikely to be due myosarcoma, and nasopharyngeal carcinoma. on September 27, 2021 by guest. Protected copyright. to malignancy. Attention should be made to associated rashes (exanthemata), travel, and exposure to pets (particularly cats). Associated Generalised lymphadenopathy constitutional symptoms should be sought: The presence of generalised lymphadenopathy weight loss (> 10% over six months), fever and should always alert the clinician to the night sweats, pruritis, and myalgia/arthralgia. presence of significant pathology. Detailed While the presence of such symptoms is history and examination will help narrow important, they are not specific, as frequently diagnostic possibilities. Any of the common believed, for lymphoma. Only one third of viral exanthemata may produce generalised children with Hodgkin's disease and 10% lymphadenopathy, other infective causes may with non-Hodgkin's lymphoma display con- include infectious mononucleosis, toxoplas- stitutional symptoms. mosis, and HIV infection particularly if lym- phadenopathy has been present for over three months. Lymphadenopathy due to leukaemia Physical examination may be generalised, but other features such as Department of While the child with isolated lymphadenopathy anaemia, bleeding, or bruising are likely to be Oncology, associated with erythema, tenderness, and present. Similarly generalised lymphadeno- Birmingham inflammation poses little diagnostic dilemma, pathy from other systemic malignancies such Children's Hospital, the non-inflamed node is more of a as lymphoma or neuroblastoma are likely to be Ladywood Middleway, chronic, Birmingham challenge. The first point is to decide whether associated with other clinical findings (for B16 8ET nodes are abnormally enlarged. Unfortunately example the presence of an abdominal mass). in the literature to A number of rarer causes of Correspondence to: there is little guidance help. generalised Dr Morland. While some authors suggest lymph nodes lymphadenopathy should also be considered in Lymphadenopathy 477 the list of differential diagnoses. A wide range biopsies in children reported about one sixth of of autoimmune and connective tissue disease cases being due to malignancy.'2 Of 13 malig- may be associated with generalised lympha- nant cases in this report, nine were due to Arch Dis Child: first published as 10.1136/adc.73.5.476 on 1 November 1995. Downloaded from denopathy. Drugs (particularly phenytoin and Hodgkin's disease, three non-Hodgkin's lym- carbamazepine) are reported to be associated phoma, and one Langerhans' cell histiocytosis. with lymphadenopathy, but this complication Although other malignancies such as soft tissue appears to be very rare.4 X linked lymphopro- sarcomas may involve locoregional nodes, it is liferative disease is characterised by a rapidly uncommon for lymphadenopathy to be present progressive and often fatal Epstein-Barr virus without additional physical signs due to the infection mimicking severe infectious mono- primary tumour. nucleosis.5 The Sezary syndrome is a form of cutaneous T cell non-Hodgkin's lymphoma with similarities to mycosis fungoides.6 Rarer pathologies Although more typically a disease of older A seemingly bewildering assortment of reactive patients, it has been reported to occur in lymphadenopathies, lymphadenitides, and lym- children and is associated with generalised phoproliferative disorders underlies a small lymphadenopathy. proportion of children with enlarged lymph nodes.'3 A few warrant further discussion as confusion with malignant nodes is not Regional lymphadenopathy uncommon.14 Due to the anatomical arrangement of the Rosai-Dorfmann disease (sinus histiocytosis lymphatic system and drainage, regional with massive lymphadenopathy) 15 may present lymphadenopathy usually represents the with alarming, bulky, and often matted lymph clinical manifestation of local pathological nodes. The majority of cases present with processes. Infection is undoubtedly the com- cervical lymphadenopathy, although other monest cause of localised lymphadenopathy, nodal sites and extranodal involvement (skin, and careful examination of the skin, ear, nose, soft tissues, bone, central nervous system) can and throat, teeth, scalp, etc will help define the be seen. Although the disease is usually seen in source of infection in a proportion of children. young adults, cases in children have been Common infecting organisms will be strepto- reported.16 Constitutional symptoms such as coccal and staphylococcal species.7 Axillary fever and malaise may be present in a propor- and cervical lymphadenopathy should always tion of cases. Common laboratory findings are prompt questioning about pets, as cat scratch raised erythrocyte sedimentation rate (ESR), disease is commonly associated with this hypergammaglobulinaemia, and anaemia. pattern of lymph node enlargement.8 Long Although the lymph nodes undergo spon- standing lymphadenopathy may suggest tuber- taneous regression with time, mortality from culosis and a history of contact should be progressive disease in vital sites is recognised.'7 sought. Tuberculin skin testing may help in A variety of therapeutic interventions have clarification, however, both false positive and been tried in order to halt the progression of false negative results may lead to confusion.9 this disease including corticosteroids, a variety http://adc.bmj.com/ In developed countries, lymphadenopathy of chemotherapeutic agents, immunosuppres- due to mycobacteria is likely to be non- sants such as cyclosporin, and radiotherapy. tuberculous and one recent report has suggested All have been shown to have some effect in the incidence of Mycobacterium avium and individual patients, but at best the responses Mycobacterium malmoense infections in children are variable, and no consistent approach can is increasing.10 The cervical, submandibular, be recommended other than observation in the and preauricular nodes are usually involved, majority of cases. A small number of cases will on September 27, 2021 by guest. Protected copyright. and in most cases the lymphadenopathy is uni- develop persistent, non-progressive disease, or lateral. Systemic illness appears to be unusual. demonstrate a chronically relapsing picture Diagnosis may be made by Mantoux testing over several years. with different mycobacterium species antigens, Kikuchi's disease (histocytic necrotising however, results may not be conclusive. In addi- lymphadenitis) was first described in Japan but tion cross reactivity with tuberculin purified is now recognised to occur world wide,'8 protein derivative
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