Presentation on theme: "Soheir Adam , MD, MSc, MRCPath"— Presentation transcript:
1 Soheir Adam , MD, MSc, MRCPath LymphadenopathySoheir Adam , MD, MSc, MRCPath
2 The Lymphatic SystemThe body has approximately 600 lymph nodes, but only those in the submandibular, axillary or inguinal regions may normally be palpable in healthy people.1 Lymphadenopathy refers to nodes that are abnormal in either size, consistency or number. There are various classifications of lymphadenopathy, but a simple and clinically useful system is to classify lymphadenopathy as "generalized" if lymph nodes are enlarged in two or more noncontiguous areas or "localized" if only one area is involved.
3 Distinguishing between localized and generalized lymphadenopathy is important in formulating a differential diagnosis.In primary care patients with unexplained lymphadenopathy, approximately 3/4 of patients will present with localized lymphadenopathy and 1/4 with generalized lymphadenopathy.
5 LympahdenopathyFindings from a Dutch study revealed a 0.6% annual incidence of unexplained lymphadenopathy in the general population.Of 2,556 patients in the study who presented with unexplained lymphadenopathy to their family physicians, 256 (10 %) were referred to a subspecialist and 82 (3.2 %) required a biopsy, but only 29 (1.1 %) had a malignancy.
8 HistoryFirst, are there localizing symptoms or signs to suggest infection or neoplasm in a specific site?Second, are there constitutional symptoms such as fever, weight loss, fatigue or night sweats to suggest disorders such as tuberculosis, lymphoma, collagen vascular diseases, unrecognized infection or malignancy?
9 HistoryThird, are there epidemiologic clues such as occupational exposures, recent travel or high-risk behaviors that suggest specific disorders?Fourth, is the patient taking a medication that may cause lymphadenopathy? Some medications are known to specifically cause lymphadenopathy (e.g., phenytoin ), while others, such as cephalosporins, penicillins or sulfonamides, are more likely to cause a serum sickness-like syndrome with fever, arthralgias and rash in addition to lymphadenopathy.
10 Medications That May Cause Lymphadenopathy Allopurinol (Zyloprim) Atenolol (Tenormin) Captopril (Capozide) Carbamazepine (Tegretol) Cephalosporins Gold Hydralazine (Apresoline)Penicillin Phenytoin (Dilantin) Primidone (Mysoline) Pyrimethamine (Daraprim) Quinidine Sulfonamides Sulindac (Clinoril)Adapted with permission from Pangalis GA, Vassilakopoulos TP, Boussiotis VA, Fessas P. Clinical approach to lymphadenopathy. Semin Oncol 1993; 20:
11 Physical Examination Size. Pain/Tenderness :The presence or absence of tenderness does not reliably differentiate benign from malignant nodes.Consistency: Stony-hard nodes are typically a sign of cancer, usually metastatic. Very firm, rubbery nodes suggest lymphoma. Softer nodes are the result of infections or inflammatory conditions. Suppurant nodes may be fluctuant. The term "shotty" refers to small nodes that feel like buckshot under the skin, as found in the cervical nodes of children with viral illnesses.
12 Physical ExaminationMatting : can be either benign (e.g., tuberculosis, sarcoidosis) or malignant (e.g., metastatic carcinoma or lymphomasLocation : infectious mononucleosis causes cervical adenopathy and a number of sexually transmitted diseases are associated with inguinal adenopathy
13 Physical ExaminationSupraclavicular lymphadenopathy has the highest risk of malignancy, estimated as 90 percent in patients older than 40 years and 25 percent in those younger than age.Lymphadenopathy of the right supraclavicular node is associated with cancer in the mediastinum, lungs or esophagus.The left supraclavicular (Virchow's) node receives lymphatic flow from the thorax and abdomen, and may signal pathology in the testes, ovaries, kidneys, pancreas, prostate, stomach or gallbladder. Although rarely present
14 Evaluation of Suggestive S & S Associated with Lymphadenopathy TestAssociated findingsDisorderEvaluation of Suggestive S & S Associated with LymphadenopathyMononucleosis-type syndromesFatigue, malaise, fever, atypical lymphocytosisEpstein-Barr virus*Splenomegaly in 50% of patientsMonospot, IgM EA or VCAToxoplasmosis*80 to 90% of patients are asymptomaticIgM toxoplasma antibodyCytomegalovirus*Often mild symptoms; patients may have hepatitisIgM CMV antibody, viral culture of urine or bloodInitial stages of HIV infection*"Flu-like" illness, rashHIV antibodyCat-scratch diseaseFever in one third of patients; cervical or axillary nodesUsually clinical criteria; biopsy if necessaryPharyngitis due to group A streptococcus, gonococcusFever, pharyngeal exudates, cervical nodesThroat culture on appropriate mediumTuberculosis lymphadenitis*Painless, matted cervical nodesPPD, biopsySecondary syphilis*RashRPRHepatitis B*Fever, nausea, vomiting, icterusLiver function tests, HBsAg
15 Lymphogranuloma venereum Tender, matted inguinal nodesSerologyChancroidPainful ulcer, painful inguinal nodesClinical criteria, cultureLupus erythematosus*Arthritis, rash, serositis, renal, neurologic, hematologic disordersClinical criteria, antinuclear antibodies, complement levelsRheumatoid arthritis*ArthritisClinical criteria, rheumatoid factorLymphoma*Fever, night sweats, weight loss in 20 to 30% of patientsBiopsyLeukemia*Blood dyscrasias, bruisingBlood smear, bone marrowSerum sickness*Fever, malaise, arthralgia, urticaria; exposure to antisera or medicationsClinical criteria, complement assaysSarcoidosisHilar nodes, skin lesions, dyspneaKawasaki disease*Fever, conjunctivitis, rash, mucous membrane lesionsClinical criteria
16 Lyme disease* Measles* Rubella* Tularemiala* Brucellosis* Plague Less common causes of lymphadenopathyLyme disease*Rash, arthritisIgM serologyMeasles*Fever, conjunctivitis, rash, coughClinical criteria, serologyRubella*RashTularemiala*Fever, ulcer at inoculation siteBlood culture, serologyBrucellosis*Fever, sweats, malaisePlagueFebrile, acutely ill with cluster of tender nodesTyphoid fever*Fever, chills, headache, abdominal complaintsStill's disease*Fever, rash, arthritisClinical criteria, antinuclear antibody, rheumatoid factorDermatomyositis*Proximal weakness, skin changesMuscle enzymes, EMG, muscle biopsyAmyloidosis*Fatigue, weight lossBiopsy*--Causes of generalized lymphadenopathy.EA=early antibody; VCA=viral capsid antigen; CMV=cytomegalovirus; HIV=human immunodeficiency virus; PPD=purified protein derivative; RPR=rapid plasma reagin; HBsAg=hepatitis B surface antigen; EMG=electromyelography.
17 Unexplained Lymphadenopathy Generalized Lymphadenopathyalmost always indicates a systemic disease is present, proceed with specific testing as indicated.If a diagnosis cannot be made, the clinician should obtain a biopsy of the node.The diagnostic yield of the biopsy can be maximized by obtaining an excisional biopsy of the largest and most abnormal nodeThe physician should not select inguinal and axillary nodes for biopsy, since they frequently show only reactive hyperplasia
18 Unexplained Lymphadenopathy Localized LymphadenopathyThe decision about when to biopsy is more difficult.Patients with a benign clinical history, an unremarkable physical examination and no constitutional symptoms should be reexamined in three to four weeks to see if the lymph nodes have regressed or disappeared.Patients with unexplained localized lymphadenopathy who have constitutional symptoms or signs, risk factors for malignancy or lymphadenopathy that persists for three to four weeks should undergo a biopsy.
19 Unexplained Lymphadenopathy Localized LymphadenopathyBiopsy should be avoided in patients with probable viral illness because lymph node pathology in these patients may sometimes simulate lymphoma and lead to a false-positive diagnosis of malignancy.
21 Risk factors for NHL immunosuppression or immunodeficiency connective tissue diseasefamily history of lymphomainfectious agentsionizing radiation
22 A practical way to think of lymphoma CategorySurvival of untreated patientsCurabilityTo treat or not to treatNon-Hodgkin lymphomaIndolentYearsGenerally not curableGenerally defer Rx if asymptomaticAggressiveMonthsCurable in someTreatVery aggressiveWeeksHodgkin lymphomaAll typesVariable – months to yearsCurable in most
23 Diagnosis requires an adequate biopsy Diagnosis should be biopsy-proven before treatment is initiatedNeed enough tissue to assess cells and architectureopen bx vs core needle bx vs FNA
24 Staging of lymphoma Stage I Stage II Stage III Stage IV A: absence of B symptomsB: fever, night sweats, weight loss
25 Case: M.S. 25 year old woman persistent dry cough fever, NS, weight loss x 3 monthsleft cervical lymphadenopathy (2 cm)left supraclavicular node (2 cm)no splenomegaly