Sickle Cell Information Center Guidelines

Edited by James Eckman, M.D. and Allan Platt, PA-C

Generalized lymphadenopathy is seen in a large number of systemic illnesses of all etiologies. In patients with sickle syndromes, it can occur secondary to extramedullary hematopoiesis, however, this is always a diagnosis of exclusion even if node biopsy shows some histologic evidence of marrow elements. Localized lymphadenopathy is more often with local infection or malignancy.

Present Illness. Inquire about age, location of nodes, duration, tenderness, recent infection, skin inflammation, or lesion in area, fever, night sweats, weight loss, pruritus, pets and animal exposure, pharyngitis, malaise, rash, and exposure to tuberculosis.

Past Medical History. Past exposure or treatment for sexually transmitted disease, sexual history, past surgery or blood transfusions, past travel, recent immunizations and medications, and date and result of last PPD.

Physical Examination.

- Vital Signs. Note temperature.

- General Inspection. Signs of recent weight loss.

- Skin. Do a thorough skin examination especially in an area of localized nodes. Define. location - palmar/plantar lesions, generalized, dermatome, or truncal; character - vesicular, macular/papular lesions, follicular, or papulosquamous.

- Head/Scalp. Scalp lesions, dermatitis, or temporal wasting.

- Eye. Jaundice, conjunctivitis, periorbital edema.

- Mouth/Throat. Palatine petechiae, pharyngitis, tonsils, thrush.

- Lymph Nodes. Characterize size, location, consistency, inflammation, fixed or non-fixed, local verses generalized lymphadenopathy

- Abdominal. Liver/spleen size and tenderness, masses.

- Extremities. Joint pain, range of motion, synovial thickening, effusions.

- Minimum Evaluation. CBC with differential, reticulocyte and platelet count, Chem profile including AST, ALT, Bilirubin, Alk Ptase, chest x-ray, PPD.

- Additional Testing. Lymph node biopsy for local nodes without infection or dermatitis, hard, rubbery, fixed nodes. PPD and control for symptoms or TB exposure. Monospot and consider EB virus, toxoplasmosis, CMV titers or cultures, HIV for mono syndrome. Hepatitis serologies if liver tests indicate. Bone marrow for change in CBC with more anemia, neutropenia, blasts, or thrombocytopnea. Erythrocyte sedimentation rate, rheumatoid factor, ANA for arthritis. VDRL/FTA, HIV if sexually active.

The evaluation of lymphadenopathy is very dependent on the age of the patient, distribution and characteristics of the lymph nodes, and associated symptoms and findings..

Localized Adenopathy

Infection and dermatitis are always considered with localized adenopathy. Location can give some clue to the etiology. Occipital - scalp inflammation/infection, rubella; Postauricular - external otitis, rubella; Preauricular - viral conjunctivitis, external otitis, cat scratch fever; Anterior cervical - infection of pharynx, tonsils, thyroiditis, lymphoma, neoplasm Submandibular/Submental - oral infection, neoplasm; Posterior cervical - scalp inflammation, mono, HIV; Supraclavicular - neoplasm of neck, lung, esophagus, abdomen, breast, lung, lymphoma; Axillary - hydradenitis, neoplasm of lung, breast, lymphoma; Inguinal - genital infection gonorrhea, lymphogranuloma venereum , Herpes, syphilis, chlamydia, neoplasm, lymphoma.

Generalized adenopathy can be seen in normal individuals with sickle syndromes and with systemic infection, collagen vascular disease, and malignancy. Age and associated clinical findings are most useful in establishing differential diagnosis.

- Normal Patient. Diffuse adenopathy is common in children, adolescents, and young adults with sickle syndromes. Hepatomegaly and splenomegaly are also common findings in sickle syndromes. Very young children and older adults do not usually have adenopathy. Systemic symptoms are absent and nodes are non-tender, discrete, and mobile.

- Viral exanthems. Many of the childhood viral illnesses which present with erythematous rashes cause diffuse adenopathy. Location of nodes and skin rash may establish the diagnosis.

- Infectious Mononucleosis Syndrome. Pharyngitis, fatigue, rash (especially with ampicillin, diffuse, tender adenopathy with posterior cervical nodes, increasing hepatosplenomegaly, and reactive lymphocytosis are seen with EB virus infection. Monospot is diagnostic but may be negative in young children and early in the infection. Monospot negative mono syndrome is seen with toxoplasmosis, CMV, and acute HIV infection.

- Acute Hepatitis. Acute viral hepatitis may present with diffuse adenopathy, fever, atypical lymphocytes, and arthralgias/arthritis. Liver tests will suggest the diagnosis.

- Sexually Transmitted Diseases. Sexually active adolescents and adults with syphilis,chlamydia, and HIV infection often present with diffuse adenopathy with or without systemic symptoms. Serologies will establish the diagnosis.

- Drug Reactions. Dilantin can cause diffuse adenopathy. Drug reactions or serum sickness from recent immunizations or medications are associated with diffuse adenopathy.

- Sarcoidosis. Diffuse adenopathy with hilar adenopathy, uveitis, pulmonary infiltrates and anergy are characteristic in sarcoidosis.

- Tuberculosis. Disseminated TB presents with fever, cough, night sweats, and weight loss. PPD may be negative in children.

- Leukemia/lymphoma. Acute leukemia in children and adults, chronic leukemia in adults, and lymphomas can present with diffuse adenopathy. Bleeding, bruising, and petechiae may be presenting complaints. Alterations in white count and platelet count may be characteristic. Bone marrow examination or lymph node biopsy with immunophenotyping will usually be diagnostic.

- Collagen Vascular Diseases. Juvenile or adult rheumatoid arthritis, systemic lupus erythematosis, and related syndromes can present with diffuse adenopathy. Joint findings are usually characteristic. Appropriate serologies will usually establish the diagnosis.

- Hyperthyroidism. lymph node enlargement is seen with hyperthyroidism.

- Mucocutaneous Lymph Node Syndrome. Children with Kawasaki’s disease present with cervical adenopathy, fever, conjunctivitis, inflamed mucosa, truncal rash involving palms and soles with desquamation of skin over tips of fingers and toes.

Treatment

Treatment in most illnesses involving lymph nodes usually does not present unusual problems in patients with sickle syndromes. There are special considerations in treating malignancies with chemotherapy where aggressive transfusion support may be required because of the suppression of erythropoiesis and the short red cell survival. Allogeneic bone marrow transplantation should be considered early in the treatment of leukemias and lymphomas in children and young adults because of the potential to cure the malignancy and sickle syndrome.

Nursing Considerations

 Provide Education on nutritious diet (high protein) and adequate amount of fluid.

 Encourage for uninterrupted periods of rest and sleep.

 Stress importance of recommended immunizations including yearly flu and pneumonia vaccines.

Teach and encourage safe sex practices to sexually active patients.

 Most community health departments will supply, free condoms yearly, gynecologic exams for females and annual PPDs.

 Offer appropriate counseling and reassurance.

Prevention

Prevention of lymphadenopathy associated diseases is primarily related to the prevention of infections. Immunizations are indicated. Contacts of active tuberculosis patients should be treated and monitored in an appropriate manner based on age. Sexually active adolescents and young adults should be taught the principles of "safe sex".

Patient and Parent Education

Patients and parents may need reassurance about diffuse adenopathy which is normal or related to viral infections. Parents need to be taught the importance of immunization for viral infections. Sex education needs to include specific information about avoiding exposure to sexually transmitted diseases.

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Greenfield S, Jordan MC. The clinical investigation of lymphadenopathy in primary clinical practice. JAMA 240:1388, 1978.

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Rosen PJ Hodgkin's Disease and Malignant Lymphoma in Manual of Clinical Oncology Casciato DA Lowitz BB editors, Lippincott Williams Wilkins 1995.

Fletcher RH Lymphadenopathy in Manual of Clinical Problems in Adult Ambulatory Care Dornbrand L,. Hoole AJ and Fletcher RH editors.Lippencott Williams and Wilkins 1998.

Libman H. Generalized lymphadenopathy. J Gen Intern Med 1987; 2:48–58.

Swartz MN. Lymphadenitis and Lymphangitis. In GL Mandell, RB Douglas Jr, JE Bennett (eds). Principles and Practices of Infectious Disease. New York: Churchill-Livingstone, 1990. Pp 818–25.

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Copyright © 1997 Sickle Cell Information Center
Last modified: October 08, 2000