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Noninfectious causes of generalized lymphadenopathy in children

Noninfectious causes of generalized lymphadenopathy in children
Condition Selected clinical features that may be present
Neoplastic
Primary
Hodgkin lymphoma Usually manifests as cervical lymphadenopathy; adenopathy may be unilateral; respiratory distress can occur
Non-Hodgkin lymphoma Rapidly enlarging diffuse adenopathy, abdominal pain, vomiting; adenopathy is usually bilateral; respiratory distress can occur
Metastatic
Acute lymphocytic or myelogenous leukemia Ill appearance, bleeding, hepatosplenomegaly, anemia, thrombocytopenia; occipital nodes often prominent
Neuroblastoma Abdominal mass; opsoclonus-myoclonus, proptosis, periorbital ecchymoses, nasal obstruction, Horner syndrome, subcutaneous nodules, secretory diarrhea
Rhabdomyosarcoma Proptosis; nasal, aural, or sinus obstruction; Horner syndrome; hematuria; urinary obstruction; constipation
Immunologic
Vasculitis syndromes (systemic lupus erythematosus, rheumatoid arthritis) Patients may have generalized adenopathy during the acute phase of illness
Serum sickness Rash, splenomegaly, myalgia, arthritis
Autoimmune hemolytic anemia Lymphadenopathy coincides with hemolysis
Chronic granulomatous disease Recurrent infection, skin abscesses, suppurative adenitis
Metabolic
Gaucher disease Hepatosplenomegaly, anemia, thrombocytopenia, osteopenia
Niemann-Pick disease Hepatosplenomegaly, loss of neurologic function
Drugs
Phenytoin, phenobarbital, carbamazepine, isoniazid, aspirin, barbiturates, penicillin, tetracycline, iodides, sulfonamides, allopurinol, and phenylbutazone Severe maculopapular rash, fever, hepatosplenomegaly, jaundice, anemia, leukopenia, and plasmacytosis occurring during or after the lymphadenopathy
Miscellaneous
Sarcoidosis Multisystem granulomatous disease; generalized adenopathy with prominent cervical involvement
Hemophagocytic lymphohistiocytosis Fever, hepatosplenomegaly, neurologic symptoms, rash
Castleman disease Fever, hepatosplenomegaly, polyclonal hypergammaglobulinemia
Langerhans cell histiocytosis Rash (brown to purplish papules), mucosal lesions, lytic bone lesions, proptosis, diabetes insipidus
Kikuchi-Fujimoto disease Cervical and supraclavicular adenopathy, fever, fatigue, weight loss, anemia, and leukopenia
Rosai-Dorfman disease (sinus histiocytosis with massive lymphadenopathy) Chronic bilateral cervical adenopathy is characteristic; other nodal groups are involved in most cases; fever, anemia, leukocytosis, elevated ESR, and hypergammaglobulinemia
Hyperthyroidism Tachycardia, hypertension, diaphoresis, weight loss, goiter, hyperreflexia
Papular acrodermatitis (Gianotti-Crosti syndrome) Rash on face, buttocks, limbs, palms, and soles; hepatomegaly
Progressive transformation of germinal centers Found in 10% of nodes with reactive hyperplasia; sometimes associated with prior diagnosis of Hodgkin lymphoma and rarely before the diagnosis of Hodgkin lymphoma
ESR: erythrocyte sedimentation rate.
Data from:
  1. Malley R. Lymphadenopathy. In: Textbook of Pediatric Emergency Medicine, 5th ed, Fleisher GR, Ludwig S, Henretig FM (Eds), Lippincott Williams & Wilkins, Philadelphia 2006. p.421.
  2. Scagni P, Peisino MG, Bianchi M, et al. Kikuchi-Fujimoto disease is a rare cause of lymphadenopathy and fever of unknown origin in children. J Pediatr Hematol Oncol 2005; 27:337.
  3. Osborne BM, Butler JJ, Gresik MV. Progressive transformation of germinal centers: Comparison of 23 pediatric patients to the adult population. Modern Pathology 1992; 5:135.
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