Lymphadenopathy Lymphoma

General information on lymphomas

Lymphoma is a general term used to describe a heterogeneous group of cancers arising from lymphocytes, key cells of the immune system. The organs most affected by the lymphomatous process are usually the lymph nodes, which can be regarded as organized accumulations of lymphocytes present in the body. Lymph nodes, the disease can spread through blood and / or lymphatic vessels to lymph nodes or other organs, and lymphatic (marrow, spleen, etc.). Extralymphatic or (skin, lungs, central nervous system, stomach, liver etc.).. Lymphocytes, in fact, even if they form and mature in the lymphatic organs, are responsible for physiological circulate in the blood and in all other organs of the body in search of foreign antigens to be deleted. The tumor cells usually do not lose this ability to circulate in the body, although they often lose their functions, thus spreading the disease at a distance of place of origin.


Types of lymphoma

Lymphomas are divided historically into two main categories: non-Hodgkin's lymphoma and Hodgkin lymphoma. These are characterized by the presence of Reed-Sternberg cells, by definition absent in non-Hodgkin's lymphomas, and are named after the doctor who will most likely first, in 1832, described the first cases of the disease. The type of lymphoma is established with histological examination under a microscope of lymph node removed by biopsy. Sometimes you must also use the biopsy of other organs, particularly the bone marrow to determine as precisely as possible the spread of lymphoma in the body or, in technical terms, to make the staging of the lymphoma itself.
The type and stage of lymphoma significantly affect the prognosis and therefore the type and duration of therapy, which is different not only in the two categories of lymphomas, but also among the many subtypes of NHL, as we shall see.
The clinical symptoms, procedures of diagnosis and staging are very similar in the two categories of lymphomas, and will be treated together. Apart from the histological classification are explained, the systems for staging and treatment of various types of lymphoma.

More than two thirds of patients with non-Hodgkin's lymphoma presents with a persistent peripheral lymphadenopathy painless. At the time of onset, the differential diagnosis of a generalized lymphadenopathy requires the exclusion of possible infectious etiologies caused by bacteria, viruses (eg., Infectious mononucleosis, cytomegalovirus infections and HIV) and parasites (toxoplasmosis). The common opinion is that a fixed node larger than 1 cm that is not associated with documented infection and persists for more than four weeks should be biopsied. However, in many histopathological subtypes of non-Hodgkin's lymph nodes often spontaneously increase and decrease in size. Thus, an incomplete regression does not exclude the diagnosis of non-Hodgkin's lymphoma. In adolescents and young adults deserve significant consideration in the differential diagnosis of infectious mononucleosis and Hodgkin's disease. There are many clinical findings which point to a diagnosis of NHL. The involvement of Waldeyer's ring, and mesenteric lymph nodes epitrochlear is seen more frequently in patients with non-Hodgkin's lymphoma than in those with Hodgkin's disease. Unlike patients with Hodgkin's disease, showing weight loss, fever or night sweats, less than 20% of patients with non-Hodgkin's lymphoma has a systemic symptoms. Systemic symptoms are more common in patients with diffuse aggressive histologies, especially in those with liver involvement and extranodal. Onset of symptoms are less frequent fatigue, malaise, and pruritus, occurring in less than 10% of patients.

The non-Hodgkin's lymphomas are also presented with chest symptoms, abdominal and / or extranodal. Although far less frequently than in Hodgkin's disease, approximately 20% of patients with NHL presented mediastinal adenopathy. These patients most often suffer persistent cough or chest discomfort, or are asymptomatic but have abnormal findings on chest radiography. Occasionally, we observe the onset of superior vena cava syndrome. The differential diagnosis of mediastinal debut include infections (eg., Histoplasmosis, tuberculosis, infectious mononucleosis), sarcoidosis, Hodgkin's lymphoma and other cancers. The involvement of retroperitoneal lymph nodes, mesenteric and pelvic is common in most histological subtypes of non-Hodgkin's lymphoma. Unless it is massive or causing obstruction, enlarged lymph nodes in these locations usually produces no symptoms. In contrast, patients who come for medical observation of a large abdominal mass, significant splenomegaly or gastrointestinal lymphoma primitive complain about symptoms similar to those caused by other space-occupying abdominal lesions. These symptoms include chronic pain, feeling of abdominal fullness, early satiety, symptoms associated with visceral obstruction or even perforation and acute gastrointestinal bleeding. The symptoms of extranodal disease are common in some subtypes of aggressive non-Hodgkin's lymphomas, but they are uncommon in lymphomas Development sluggish. Rarely, patients present with symptoms of unexplained anemia. Patients with aggressive non-Hodgkin's lymphoma may present initially with skin lesions, testicular masses, acute compression of the spinal cord, solitary bone lesions and, rarely, meningitis linfornatosa. The primary non-Hodgkin's lymphoma of the central nervous system is only l% of all non-Hodgkin's lymphomas. However, with the increased incidence of infection with HIV-1, as well as the increased use of high-dose immunosuppressive therapy, lymphoma, primary central nervous system has become as common as glioblastoma multiforme.

When the NHL is present at extranodal, the differential diagnosis is more difficult. Rarely, patients with non-Hodgkin's lymphoma presenting with paintings of pulmonary involvement in character linfoangitico bronchovascular, nodular or cellular. Is rather frequent infiltration of the liver, although often not apparent clinically. It presents with hepatic infiltration 25-50% of patients with non-Hodgkin's lymphoma, although relatively few have large liver mass. Nearly 75% of patients with lymphoma development has advanced in torpid liver microscopic infiltration at presentation. In contrast, the primary hepatic lymphoma is extremely rare and is almost always a histologically aggressive tumor. Another debut extranodal localization, which occurs in less than 5% of patients, primary bone lymphoma. This is almost always of B-cell lymphoma, diffuse large cell, which is presented with a painful bone site. Very often, an X-ray bone osteolytic lesions are observed. The most common sites of primary lymphoma of bone is the femur, pelvis and vertebrae. About 5% of non-Hodgkin's lymphoma occurs as a primary lymphoma in the gastrointestinal tract. These patients have bleeding, pain, or obstruction, in descending order of frequency, since it has the stomach to be more often infiltrated, followed respectively by the small intestine and the colors. Most gastrointestinal lymphomas had histological features of aggressive dissemination, with a subgroup consisting of low-grade lymphomas (MALT lymphomas). Uncommon onset of non-Hodgkin lymphoma and renal infiltration is even less common is the onset of localized prostate, testis or ovary. Another common site of presentation is not localized infiltration of the skin, usually from a histologically aggressive lymphoma, the onset central nervous system is relatively rare, mainly represented by lymphomas, primary brain and spinal cord. The main central nervous symptoms include headache, lethargy, focal neurological symptoms, seizures or paralysis. Rare primary sites of lymphoma are the orbits, heart, breast, salivary glands, thyroid and adrenal glands.
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STAGING AND DETECTION OF DISEASE

The Ann Arbor staging system, developed for Hodgkin's disease, was also used in the staging of non-Hodgkin's lymphomas. This staging system is based on the number of tumor sites (nodal or extranodal), location and the presence or absence of systemic symptoms. The table summarizes the staging system. In stages I and II disease of the premises on the same side of the diaphragm are affected. The stage III disease involves both sides of the diaphragm, while stage IV is defined as extranodal lymphomatous involvement, most of the bone marrow and liver. Systemic symptoms (fever, weight loss greater than 10% of body weight and night sweats, ie, B symptoms) are less common in non-Hodgkin's lymphomas (manifesting itself in about 20% of cases) than in Hodgkin's disease. The scheme was created specifically for staging Hodgkin's disease, which spreads through the lymphatic structures and adjacent lymph only very minor for hematogenously. Since non-Hodgkin's lymphomas mainly by hematogenous spread, this staging system has proven less useful in this type of lymphoma.

The first stage: Involvement of a single lymph node region or a single site extralymphatic

First stage II: Involvement of two or more lymph node regions on the same side of the diaphragm, involvement of one site located adjacent extralymphatic and a lymph node region (stage IIE)

First stage III: Involvement of lymph node regions on both sides of the diaphragm, including the spleen can be

First stage IV: Involvement of one or more bodies littered extralymphatic with or without lymph node involvement

In patients with non-Hodgkin's lymphomas, the concept of staging has little relevance on the therapeutic strategy. Only 10% of patients with follicular lymphoma have localized disease and is a candidate for local radiotherapy. As for the aggressive diffuse lymphoma, even patients with localized disease are currently undergoing a systemic treatment. Thus, in non-Hodgkin's lymphoma staging is carried out to identify those few patients who may be treated with local therapy and to identify prognostic subgroups within the histological subtypes.

Staging procedures after diagnostic biopsy.

Staging must be done with due regard to histology. One type of proposed staging for patients with non-Hodgkin's lymphoma is the following:

Tests for the staging of non-Hodgkin's lymphoma

Essential

l. Pathologic documentation collected by a emopatologo

2. Physical examination

3. Documentation of B symptoms

4. Laboratory investigations

a. Haematology

b. Liver function tests (including the measurement of LDH)

c. Renal function test d. Uric acid

e. Football

f. Electrophoresis sieroproteica

g. serum beta 2 microglobulin

5. Radiography of the chest

6. TC addominopelvica

7. Biopsy procedures

Essential in certain circumstances

1. CT of the chest

2. CT of the head

3. Lumbar puncture

4. Barium studies of gastrointestinal canal

5. Endoscopy

6. Cytological examination of the payments cavity

7. Gallium scintigraphy (planar or SPECT) is useful but not essential

1. Phenotypic analysis of cell surface markers

2. Cytogenetic analysis

3. Analysis of gene rearrangements

4. Analysis by PCR for the presence of minimal residual disease

5. Flow cytometry for analysis of DNA

6. Liver scintigraphy

7. Liver biopsy

8. Bone scan

g. Thallium scintigraphy

10. Lymphography

11. Echography

12. Magnetic resonance imaging

13. Echocardiogram

14. Laparotomy


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