A- Definition and positive diagnosis of superficial lymphadenopathy:
It is the increase in the volume of a lymph node that becomes palpable. This node may sit in one of the following areas:
– Cervical (submandibular, submental, tragus, occipital, carotid-jugular, supraclavicular, rétrospinale);
– Inguinal and rétrocrurale;
– Épitrochléenne, popliteal, intercostal.
B- Differential diagnosis:
1- Nature or not lymph node swelling:
Whatever the nodal area must be removed a lipoma, a non-nodal malignancy, benign tumors (fibroma, neuroma). In the cervical areas should be rejected hypertrophy of a submandibular gland (a bidigitale palpation) or parotid (sometimes seat of a intraparotid lymphadenopathy); thyroid tumor (mobile swallowing); a mass of vascular origin (swing if it is as a carotid artery aneurysm) or vestigial thyroglossal duct cyst. The axillary areas may be the seat of a hidradenitis (inflammation of a sweat gland). In inguinocrurales areas must be removed hernia (impulsive cough), phlebitis of the arch of the saphenous (indurated cord), an arterial aneurysm (swing mass expansive).
2- nodes palpable but not pathological:
In children and lean subject, it is not uncommon to feel small nodes (less than 0.5 cm) particularly in the cervical and inguinal areas. When in doubt about a pathological aspect, monitoring is essential in any way.
C- Elements of the etiologic diagnosis:
It allows to specify the date and circumstances of discovery, evolutionary terms, painful or not the lymphadenopathy.
General signs Wanted: fever, night sweats, fatigue, anorexia, weight loss, generalized pruritus. Personal history will also be specified: alcohol and tobacco ground, unprotected sexual contact, TB contagion, contact with animals (cat scratch, bite), taken medication, travel and family history (including cancer).
2- Clinical examination:
• It must methodically examine all lymph nodes to distinguish:
– Isolated lymphadenopathy: lymphadenopathy one or a group of lymph nodes in one lymph node sitting area;
– The polyadenopathies, sitting in several lymph nodes, which can be symmetrical or asymmetrical.
• Clarify the physical characteristics of these lymph nodes:
– Location and size (measured best with a caliper); – Consistency: soft, elastic, rénitente, hard, sometimes stony;
– Existence of tenderness;
– Inflammatory aspect, red, hot, fluctuating or even “présuppuratif”;
– Mobility and adherence to deep planes.
• It must of course perform a complete physical examination and search specifically:
– Splenomegaly and tonsillar hypertrophy;
– A lesion in the drainage area of a single lymph node;
– Indirect signs indicating the presence of deep lymph nodes. It is basically a superior vena cava syndrome (eyelid edema, facial and supraclavicular, jugular turgor, chest collateral circulation) and edema of the lower limbs or compression of thrombotic complications.
• It is imperative to refer these findings on a dated scheme.
• Some are simple and systematic. These are: blood counts that can bring information Capital: cytopenia, polynucleosis, mononucleosis, presence of atypical lymphoid cells, or blasts, eosinophilia …; looking for inflammation (erythrocyte sedimentation rate, C-reactive protein).
• Others are undertaken only to the context: chest X-ray that can detect mediastinal lymphadenopathy or lung nodules (CT is the gold standard for the detection of deep lymph nodes); electrophoresis of serum proteins seeking a monoclonal peak; LDH (lactic dehydrogenase) (tumor mass tag lymphoma); viral serologies (including serology human immunodeficiency virus); ganglionic puncture; lymph node biopsy.
Based on the information obtained during this first step, diagnostic guidelines are different in the case of an isolated lymphadenopathy or lymphadenopathy, and if the occurrence of context is acute or chronic.
A- isolated lymphadenopathy:
To say that is isolated lymphadenopathy involves, as we have seen that all the lymph nodes were examined and a splenomegaly was sought. The diagnostic approach will rely mainly on the speed of evolution, the physical, very careful examination of the territory drainage of the lymph node, and often the first ganglion by puncture or biopsy.
1- Acute Evolution:
This is usually an infection in the territory of the lymph node drainage. The diagnosis is easy in front of a clearly inflammatory lymphadenopathy: the pressure to pain, heat, redness, or even softening periadenitis and fistula, sometimes with general signs (fever).
• infectious adenitis “pyogenic” is the most common. The focus of infection in the drainage area is not always found.Lymphadenitis may be associated with lymphangitis. Whatever the range, it may be a skin infection. Depending on the location, we search:
– High cervical: dental focus of infection, facial, ocular, oropharyngeal which angina, otitis;
– Tragus: eye infection;
– Axillary: upper limb injuries, especially the fingers (paronychia);
– Inguinal-femoral: lesions of the lower limb (erysipelas, paronychia). The lymph node puncture is often suggestive by the presence of pus that must be drawn for cytological and bacteriological study. It should be noted that the puncture of a thyroglossal duct cyst brings back a purulent liquid; restores cytology diagnosis.
• Other causes are to be found in context:
– Acute diseases inoculation follow bites, wounds, animal scratches the territory drainage: it is essentially pasteurellosis (very painful wound recent few hours with serous discharge, lymphangitis, rapid changes), and tularemia (rodent bite which hare incubation 4-8 days);
– Venereal diseases are referred to the notion of unprotected sex, genital ulcers, anal or pharyngeal. Indeed, some of these conditions may be associated with acute lymphadenopathy evolution herpes and chancroid (Haemophilus ducreyi). In this situation, serology of human immunodeficiency virus and the search for gonococcal are systematic.
– Lymphomatous lymphadenopathy can sometimes mimic acute adenitis. The lymph node puncture then directs the diagnosis.
2- chronic or subacute evolution:
A lymph node metastasis or hematologic malignancies are the assumptions to consider first, even if an infectious cause is not excluded.
• We must seek a tumor in the neighborhood: it is fixed bulky lymphadenopathy and stony consistency, but these characteristics are not constant. The tumor is sometimes evident on clinical examination (or chest radiography) in the area drained by the lymph node:
– Melanoma, squamous skin cancer regardless of nodal area;
– Tumors of the upper aerodigestive tract (floor of the mouth, tongue, lips), thyroid nodule, for cervical lymphadenopathy;
– Dysphagia, mass on chest radiograph suggestive of esophageal cancer for a supraclavicular lymphadenopathy (especially right);
– Breast tumor for a supraclavicular or axillary lymphadenopathy;
– Abdominal mass, suspected liver nodules, testicular tumor, for a left supraclavicular lymphadenopathy (ganglion Troisier);
– Tumors of the penis, vulva and anal canal for inguinal or femoral lymphadenopathy. If the tumor is not evident, lymph node puncture directs the diagnosis by finding metastatic cells; it is then necessary to quickly make an assessment in the search for a primary neoplasm that will be biopsied:
– Panendoscopy upper aerodigestive tract in cases of cervical lymphadenopathy;
– Mammography if axillary lymphadenopathy or supraclavicular;
– Thoracic and abdominal CT scan, upper gastrointestinal endoscopy, colonoscopy if supraclavicular lymphadenopathy. If this balance is negative or if the tumor is easily accessible, a lymph node biopsy will be decided. For some, the puncture of a lymph node metastatic promote the local spread of the disease. Thus, if the clinical context is in favor of metastasis, stock looking for a primary lesion without recourse to puncture can be undertaken.
• Some items can towards haematological malignancies, mainly Hodgkin’s disease or non-Hodgkin lymphoma. The lymph nodes are usually firm and not painful, but their clinical characteristics are actually quite variable.
These blood diseases can also be detected before polyadenopathy. The elements moving towards a Hodgkin’s disease or non-Hodgkin lymphoma, very fickle, are:
– General signs: fever, sweating, weight loss;
– Itching especially in Hodgkin’s disease and non-Hodgkin lymphoma phenotype T;
– Ganglion pain to alcohol ingestion, very rare but highly suggestive of Hodgkin’s disease;
– Elevated LDH, aggression and tumor mass marker for malignant non-Hodgkin lymphoma (more than in Hodgkin’s disease);
– Eosinophilia in Hodgkin’s disease and certain non-Hodgkin lymphoma phenotype T;
– Atypical lymphoid cells on blood smears in non-Hodgkin lymphoma with bone marrow and blood passing invasion.The lymph node aspiration cytology can provide valuable arguments: presence of cell evoking Sternberg Hodgkin’s disease, homogeneous smear evoking a lymphoid cell non Hodgkin’s lymphoma. However, cytological analysis of lymph node smear is indicative only, and lymph node biopsy is needed to confirm any of these diagnoses. The evolution of some malignant non-Hodgkin’s high malignancy can be very fast with metabolic disorders, neuromeningeal reached, compression, and perform a diagnostic and therapeutic emergency.
• Some infectious causes may also be referred to isolated lymphadenopathy. The lymph nodes are chronic or subacute, sometimes associated with discrete symptoms (mild fever). Adenitis to mycobacteria: Mycobacterium tuberculosis (Mycobacterium tuberculosis) is the most commonly involved. We think it more likely in a patient’s native third world or from a disadvantaged background. This is usually one or more cervical lymphadenopathy, firm consistency then torpid evolution towards softening and fistula. The lymph node biopsy is required when the lymph node puncture shows a purulent fluid containing acid-fast bacilli (AFB) direct examination after Ziehl staining.
A culture on Löwenstein is essential for identification and susceptibility testing. The search for other locations is the rule, and antibiotics will be prescribed for 6-9 months. Other mycobacterial diseases are rarer. Mycobacterium bovis is rather found in the axillary locations. Atypical mycobacteria are mainly seen in immunocompromised patients. The “BCG infection” is a suppurating at the vaccine injection site associated with axillary lymphadenopathy satellite. The cat scratch disease (scratch disease) is suspected in the concept of scratch, sometimes old (incubation 7-60 days) by a cat. The lymphadenopathy, weakly inflammatory, slowly evolving into suppuration (Possible spontaneous regression). The lymph node puncture with Warthin-Starry stain can highlight coccobacilli. Serology Bartonella henselae and Afipia felis and especially search the bacterial genome (of lymph node removal) provide the diagnosis. The toxoplasmosis primary infection is rare as an isolated lymphadenopathy.
The lymphadenopathy is discreetly sensitive and inflammatory, usually cervical (including submental). Are rarely mentioned, following a venereal contact, Nicolas-Favre syphilis and disease as a result of dental, cervical actinomycosis.
Always seek associated splenomegaly. The main guidance before polyadenopathy are: signs suggestive of viral infection, the evolution (acute or chronic), the symmetrical distribution or not lymph nodes, blood count and serology for human immunodeficiency virus. These elements will sometimes easily lead to diagnosis, in other cases, only the puncture and lymph node biopsy will make a diagnosis.
1- Infectious causes:
• Acute Evolution: the lymph nodes are often symmetrical and small. It can be accompanied by signs of acute viral infection (general symptoms, rash, pharyngitis, tonsillitis, conjunctivitis, flu syndrome …) we must seek a concept of contagion. The existence of mononucleosis in careful examination of blood smear strongly directs the diagnosis. It is the presence of large atypical lymphocytes hyperbasophilic aspect, associated with small lymphocytes of normal appearance and the appearance of plasma cells cells. Sometimes there is a lymphocytosis. This syndrome is isolated or associated with thrombocytopenia (peripheral origin) and a discrete neutropenia. Two causes are predominant, one by its frequency (infectious mononucleosis), the other by its gravity (primary infection by the human immunodeficiency virus). Infectious mononucleosis is a quick installation infection that usually combines angina, a marked weakness, and sometimes a fever. A morbilliform rash spontaneous or triggered by penicillin A decision is very evocative. The polyadenopathy, usually posterior cervical predominance, can take a tumoral aspect.A liver enzymes is frequent. Diagnosis is based either on the detection of heterophile antibody test for infectious mononucleosis, highly sensitive (95%), but to control by the reaction of Paul-Bunnell-Davidsohn or on the presence of anti-VCA IgM (viral capsid antigen) to EBV serology. Primary infection by human immunodeficiency virus may be symptomatic. The table is close to infectious mononucleosis fever, flu syndrome, asthenia, pharyngitis, rash sometimes triggered by penicillin A moderate hepatic cytolysis. These events are transient, disappearing within weeks. We must look for risk factors. Diagnosis is based on serology of the virus at this stage can still be negative, then imposing the p24 antigen. In case of strong suspicion if p24 antigen and viral serology are negative, the study of viral load may be required. Other etiologies exist: toxoplasmosis; rubella (posterior cervical lymphadenopathy often very small); adenovirosis; primary infection with cytomegalovirus. The mononucleosis is not always present and the lymph nodes can summarize the clinical presentation; serology alone will make the diagnosis.
• Chronic Evolution: we must think first of the widespread chronic lymphadenopathy associated with human immunodeficiency virus. This polyadenopathy is symmetrical and sometimes associated with opportunistic infections. Diagnosis is based on serology of the virus. In this context, the emergence of asymmetrical lymphadenopathy, large (> 2 cm), more acute course, should suggest lymphoma associated with immunodeficiency virus. The puncture may show suspicious cells and lymph node biopsy is needed. Other infectious causes are rare, brucellosis, secondary syphilis, visceral leishmaniasis, lymphatic tuberculosis [generalized form and (or) hematopoietic].
2- malignant causes:
• Acute leukemia is evoked, especially if it is also a weakness, an infectious syndrome and purpura. The presence of blasts in the blood, regardless of the rate, with or without signs of bone marrow failure (aplastic anemia, neutropenia, thrombocytopenia) is strongly suggestive of the diagnosis. Pancytopenia without blastosis should also suggest the diagnosis of acute leukemia. This assumption must be confirmed by a myelogram emergency and requires prompt hospitalization. This is usually an acute lymphoid leukemia than myelogenous.
• The diagnosis of chronic lymphocytic leukemia (CLL) will be referred to a blood lymphocytosis (lymphocytes> 4 G / L). Examination of the blood smear shows small lymphocytes banal aspect, and altered cells (shadows Gumprecht).The lymphocytosis is accompanied by anemia and (or) thrombocytopenia in advanced forms. The polyadenopathy is symmetrical, evolving into a “lazy” context. The immunological study (markers) of lymphoid cells from peripheral blood is the key to diagnosis (B cells, CD5 +, CD23 + and low density of expression of membrane Ig).
• Other chronic lymphoid hematological, near-chronic lymphocytic leukemia, but less frequently may be raised about the presence of atypical lymphoid blood cells. There is often a hyperlymphocytosis sometimes accompanied by anemia, thrombocytopenia, or neutropenia. It can be a malignant non-Hodgkin’s lymphoma of low malignancy (follicular lymphoma, mantle cell lymphoma) with blood spread, of prolymphocytic leukemia, a disease Waldenstrom (association with a serum monoclonal IgM> 5 g / L). The cytological and immunological study of lymphoid cells from peripheral blood is still the basis of diagnosis. Then, depending on the case, a lymph node biopsy, a bone marrow aspirate, bone marrow biopsy, a karyotype of tumor cells may be requested.
• There are sometimes very suspicious signs of malignant non-Hodgkin’s lymphoma or Hodgkin’s disease particularly to asymmetric polyadenopathy with large items (> 2 cm), chronic or subacute or rarely acute course (in malignant non Hodgkin very high malignancy). The result of lymph node puncture generally strengthens the suspicion. But the lymph node biopsy is needed to confirm the diagnosis and identify the type of lymphoma.
• More rarely asymmetric polyadenopathy chronic or subacute evolution may indicate metastatic cancer. The diagnosis strongly suspected to be confirmed lymph node puncture by the discovery of a primary tumor or lymph node biopsy.
3- Other causes:
• Sarcoidosis is raised before polyadenopathy cervico-axillary, sometimes épitrochléenne. The general signs are not marked. Chest radiography is suggestive: hilar mediastinal lymph nodes, and sometimes intertrachéobronchique interstitial syndrome. Systemic involvement is possible. Diagnosis relies on the detection of a granuloma epithelioid and giant-casein without the lymph node biopsy.
• Over the systemic disease, lymph nodes where they exist are small. They are found in lupus especially, but also in rheumatoid arthritis, Still’s disease, dermatomyositis, Sjogren’s syndrome, Kawasaki disease, Whipple’s disease …
• A drug intake can induce the occurrence of lymphadenopathy (hydantoins, quinidine, beta-lactam antibiotics, nonsteroidal anti-inflammatory drugs). Nevertheless, this hypothesis will be retained after eliminating other causes.
The disappearance of these lymph nodes to drug withdrawal can be slow, but it is essential for diagnosis.
Some conditions can be sometimes referred to responsible polyadenopathies “dysimmune” mimicking any point lymphoma. This is Castleman’s disease, lymphadenopathy complicating the constitutional immunodeficiencies, and Rosai and Dorfman disease.
In these situations, only the lymph node biopsy to rule out the diagnosis of lymphoma.
Highlights to include:
• The discovery of one or more superficial lymphadenopathy is sometimes an element of obvious or known diagnosis. However, this discovery is often the starting point of the diagnostic process.
• It is important to determine the clinical characteristics of these lymph nodes: the distinction between isolated lymphadenopathy and polyadenopathy is the basis of diagnostic reasoning.
• An isolated lymphadenopathy should be investigated lesion (cancer, infection) in the drainage territory before considering any other cause.
• The polyadenopathies are multiple causes, dominated by viral infections, but they sometimes reveal a blood disease (leukemia, lymphoma).
• The lymph node puncture is a simple examination, frequently performed, which often provides diagnostic orientation, but can not replace the lymph node biopsy remains necessary to confirm the diagnosis of lymphoma or Hodgkin’s disease.
1 / Lymph node aspirate:
The puncture is done using a fine needle aspiration without. After engagement of the end of the needle into the node, lymph aspirate rises by capillarity. The content of the needle is projected onto a slide using a syringe, then spread for cytological analysis. On suspicion of purulent adenitis, sucking in the liquid which is punctured asked bacteriological analysis: Direct examination with Gram stain and Ziehl-Neelsen and culture on Lowenstein-Jensen standard environments. Other techniques may be requested depending on the clinical orientation (Warthin-Starry stain, etc.). If the smear is suspicious of lymphoma (Hodgkin’s disease or non-Hodgkin lymphoma), a lymph node biopsy is essential. If the smear is suspicious of lymph node metastasis, primary tumor is sought. If the smear is polymorphic “of reaction speed” monitoring is performed, and biopsy in a second time if there is persistence or increase volume, or an immediate biopsy if there is a large lymphadenopathy or suspicion clinical malignancy.
2 / lymph node biopsy:
She is determined:
• when no obvious cause is at the end of explorations, and that the lymphadenopathy persists after a period of several weeks of surveillance;
• When non-Hodgkin’s lymphoma or Hodgkin’s disease is likely (puncture clinic), to provide a definitive diagnosis and to specify the type of lymphoma;
• If TB is suspected (not proved by lymph node aspiration or other method);
• If the lymph node metastasis smear is suspicious and that the primary tumor is undetectable or inaccessible (for some lymph node excision is essential in any way). Precautions are necessary:
• before treatment (including corticosteroid therapy);
• must arrange for the collection can be made the histological examinations, cytological (fingerprints), immunological (immunohistochemistry and flow cytometry), cytogenetics, and possibly bacteriological and molecular biology study (prevent laboratories). Rapid transit and without fixer is imperative. At best a sample will be frozen;
• must take the entire ganglion (but cleaning is not necessary, except in certain cases of metastases): this is an excisional biopsy.
Strong Points to remember:
Always consider that the occurrence of lymphadenopathy is pathological.
• infectious and neoplastic diseases are the major causes of lymphadenopathy.
• The lymph node biopsy is an essential gesture when no etiologic diagnosis could not be made and to specify the histological type in malignant lymphomas.