LYMPHOMAS, HODGKINIENS AND NOT HODGKINIENS
1 malignant lymphomas, Hodgkin and NON Hodgkin Pr Gilles SALLES-October 2011 INTRODUCTION:
Malignant lymphomas are a group of lymph, ganglionic or extra-ganglionic tissue cancers linked to the neoplastic transformation of a lymphocyte cell. This ensemble is the largest group of malignant hemic, the most common and about 5% of adult cancers. Hodgkin's disease (MDH) and Non-Hodgkin lymphoma (NHL) are classically distinguished. These pathologies have greatly benefited from the recent advances in chemotherapy and immunotherapy in the form of Monoclonuax antibodies i-epidemiology: I-1 frequency: Malignant lymphomas account for 3 to 5% of all adult cancers. The overall annual incidence can be estimated at 10 new cases for inhabitants. This is a more common pathology in humans (60%) than in women (40%). C is the 6th cancer in terms of incidence in humans as in women in France the frequency of lymphoma has increased considerably in recent years, with an incidence multiplied about 2 in years. In children, non-Hodgkin lymphomas are much more common than Hodgkin's disease, and there are essentially high-grade lymphomas of malignancy. Most types of lymphoma occur at all ages, although some low-scaling lymphomas (low-grade malignancy or indolent) appear to be encountered more frequently in older subjects. I-2: Specific epidemiological and geographical characteristics: II-2-1-Burkitt lymphoma This aggressive lymphoma, especially encountered in children, is readily encountered in African countries, where it was first described in 195O by a English surgeon D. BURKITT. In these countries, there is a very close epidemiological relationship between NHL occurrence and Epstein Barr virus (EBV) infection. If this virus is capable in vitro of immortalizing B lymphocytes, its precise role in lymphocytic transformation remains imperfectly understood. It is likely that other factors must be associated to show Burkitt lymphoma: malaria, immunosuppression,... II-2-2-lymphoma and HIV infection the frequency of non-Hodgkin malignant lymphomas, particularly of aggressive type (immunoblastic lymphoma and Burkitt lymphoma), is strongly increased in HIV-infected individuals. The causes of this increase are at least partly related to the immune deficiency. II-2-3-lymphoma and immunosuppressive therapies
2 subjects receiving long-term immunosuppressive therapy (e.g. organ transplant recipients, anti-TNF antibodies in chronic inflammatory diseases) or subjects treated several years previously for Hodgkin's disease present a Increased risk of non-Hodgkin's malignant lymphomas. II-2-4-T-lymphoma of the adult associated with HTLV 1 There is a particular form of aggressive evolution in Japan and the Caribbean, which is readily affecting spleen and skin, called ATL (Adult T lymphoma-leukemia) which is closely associated with Infection with HTLV retrovirus 1. II-2-5-lymphomas and autoimmune pathology a number of autoimmune diseases represent a favourable terrain for the development of non-Hodgkin malignant lymphomas: Sjögren's syndrome, non-cutaneous lymphoid infiltration Specific, celiac disease... II-2-6-mucosal lymphomas and bacterial infections. Lymphomas developed in certain mucous membranes (stomach, skin, appendages of the eye) sometimes appear to develop after the presence of a chronic bacterial infection (Helicobacter pylori for the stomach, Borrelia burgdoferi for the skin, chlamydia Psittaci for the eye conjunctiva. This is especially true for small-cell stomach lymphomas that can be treated in 2/3 cases successfully by specific antibiotics against these bacteria. The precise incidence of bacterial infection and treatment activity in other forms is less well known. II-2-7-lymphomas and other viral infections. The incidence of lymphoma is increased in patients infected with hepatitis C viruses, to a lesser extent hepatitis C. In addition to the role of EBC (above) in immunocompromised patients (post-transplant, HIV, very old subjects), the HHV8 virus is associated with a particular clinical form (serous lymphomas). II-2-8-Conclusions If some of these associations allow for the development of basic research on the mechanisms of carcinogenesis of lymphoid cells and to formulate hypopathophysiologicalic hypotheses, it must be recognized that all these cases Represent only a minority of patients with malignant lymphomas. In the vast majority of patients, no favourable terrain and no particular etiology are found. The role of environmental factors is suspected (pesticides, various chemical compounds) but many epidemiological studies remain to be done. It is possible to conceive from the above models that repeated antigenic stimuli (microwells, viral or chemical antigens) result in a prolonged expansion of the immune cells promoting their transformation into cells Malignant, perhaps on particular genetic susceptibility grounds. True family forms, if they exist, are exceptional, but it is believed that there is an increase in the low but significant risk in the direct family of an individual with lymphoma.
3 II-Clinical study of the NHL II-1 discovered by a ganglionic presentation: The revelation of malignant lymphomas in the form of a ganglionic mass is the most common diagnostic circumstance. In one-third of cases, adenopathies are superficial, unique, or multiple. All ganglion territories can be reached, the size and consistency of the adenopathies are variable. It can also be deep, mediastinal or abdominal adenopathies. When these deep adenopathies are voluminous, they can be revealed by the presence of various symptoms and nonspecific: cough, pleural pain, cave compressive syndrome, abdominal pain, transit disturbances... A chest X-ray or abdominal ultrasound, as appropriate, can be used to highlight these adenopathies. Some histological forms have classical clinical presentations, which must be known:-lymphoblastic lymphoma of the young subject revealed by mediastinal masses of rapid development, causing Cave syndrome and sometimes dyspnea; -Burkitt lymphoma revealed by a voluminous abdominal mass or gingival tumor (cheek tumor of African children); -Low-evolutionary chronic adenopathies with sometimes spontaneous regressions in some low-grade follicular lymphomas. II-2 impairment of other hematopoietic organs: the revelation of malignant lymphoma or Hodgkin's disease in the form of an isolated splenomegaly is rare. A hepatomegaly can also be a diagnostic circumstance, even less common (often associated with a splenomegaly). Isolated spinal cord damage revealed by anemia or pancytopenia is also rare. Blood damage with circulating lymphoma cells is mainly found in indolent lymphomas. II-3-visceral localization: especially in non-Hodgkin lymphomas, visceral attacks can reveal the disease:-ENT injury, with enlarged tonsils, obstruction of cavum...-Digestive, with pain Epigastric, transit disturbances... Endoscopies with biopsies are then indispensable,-bone or epidurals localization, with medullary compression,-visceral attacks can also be cutaneous, cerebral, leptomeningeal, bone, gonadal, pulmonary... with the Associated clinical signs. II-4 revelation by a decline in the general state: Malignant lymphomas can be revealed by a decline in the general state with slimming and asthenia, a long-running fever, an unexplained inflammatory syndrome. It is then gladly extended forms, with medullary injury, or hepatosplenic.
4 II-5 positive diagnosis: the diagnosis of non-Hodgkin malignant lymphomas must be constantly based on histological study of the lymph nodes or tumor tissue reached. A surgical biopsy is necessary to obtain a good-sized ganglionic fragment that will allow:-a standard histological study and immunological techniques-a cytological study after ganglionic affixing to the blade,-freezing of fragments for more complete immuno-histochemical studies, even for molecular biology,-possibly a cytogenetic study. Therefore, ganglion samples must be received quickly and intact after resection in specialized laboratories. In the majority of cases, a histological confirmation by an expert pathologist is necessary, d where the interest of using outset to the specialized teams. While the cytology of the lymph nodes often allows diagnostic orientation, it usually does not allow all these studies, is only an element of orientation and the biopsy for histological study remains in all cases possible Essential. Indeed, only histological and immunological examinations allow a correct classification which can obtain a prognosis and provide the necessary indications for the adapted treatment of the patient. II-6 Differential Diagnosis: II-6-1 clinical problems in the face of superficial adenopathies, several diagnoses can be evoked: malignant lymphoma, metastasis of other types of cancer (to be inventoried according to location), but also of Various non-malignant pathologies:-Infectious, viral (infectious mononucleosis, poly-lymphadenopathy linked to HIV, CMV, rubella,...) or not (tuberculosis, toxoplasmosis, chlamydia, inflammatory reaction to a near-skin outbreak with banaous germs..). -Diseases "System ", Sarcoidosis... A simple biological assessment (VS, NFP, Serology...) and radiographic can sometimes help to clarify the diagnosis. In practice, when the lymph nodes are more than one centimetre in diameter, when there is a violation of several territories, a context of decline of the general state, a lack of a free infectious context or of contage, it is necessary to quickly resort to The biopsy. II-6-2 Pathology problems The differential diagnoses raised by pathology may be considered in several cases:-differential diagnosis between certain follicular-architecture lymphomas and hyperplasia of Germ follicles encountered in benign ganglion reaction pathologies; These sometimes difficult diagnoses underline the interest of immunological studies and molecular biology; -Differential diagnosis between certain indifferentiated carcinomas and large cell anaplastic lymphomas: The immuno-histological study allows to link the malignant proliferation to a lymphomateuse pathology, and to start a Effective treatment
5-Differential diagnosis of lymphoblastic lymphoma or Burkitt lymphoma with medullary invasion and lymphoblastic or LAL 3 leukemia with ganglion masses; The presence of a pancytopenia, lymphoblastic cells in the blood, or in large amounts in the marrow (> at 30%) usually lead to treating these patients as acute enzootic lymphoblastic. III-Extension balance and PRE-therapeutic balance: An extension balance is always required in malignant lymphomas. It must be able to bring together the main prognostic elements to choose a treatment adapted to the case of the patient. It must not, however, delay the treatment for too long and unnecessarily. III-1 Clinical Extension: The clinical examination allows to assess the number of ganglion territories reached, and the search for certain visceral locations (ENT). The presence or absence of general signs (slimming of 10% or more of the body weight, presence of unexplained fever for 15 days, night sweats forcing the patient to change at night) must be clarified by the interrogation. Finally, the patient's age and general condition are important prognostic elements. III-2 radiological examinations: The Making of a pulmonary radio is in all cases indispensable. The thoracic scanner provides information on the existence of a mediastinal or pleural injury. The abdominal and pelvic scanner (rather than ultrasound) is for the presence of lumbosacral-aortic and iliac adenopathies, splenic and hepatic hiles, or visceral masses. Other radiological explorations can be envisaged according to certain specific locations: cerebral scanner, ENT scanner, MRI, bone radios... More recently, functional imaging (positron emission tomography or PET) appeared with 18-fluoro-Deoxyglucose (18 FDG) scans. This tracer is attached to metabolically active cells with increased glucose uptake, which is the case for tumor lymphoid cells. Despite the presence of possible false positives (infections, recent scar, hyperthyroidism,), this PET examination at 18 FDG allows to detect ganglion and visceral locations of the infra-clinical disease, or to specify the specificity of images put Highlighted by other exams (scanner, ECHO, etc.). Technically, this examination can be carried out with a scanner (which must be carried out after injection of contrast output) in some centers. The use of this examination becomes increasingly common to the diagnosis, and it has become indispensable to evaluate the response to the treatment. It should not be done as a long-term follow-up review. III-3 Biological examinations: A formula count will always be carried out which will specify the possible existence of anemia or other cytopenia and on which the presence of possible blood flooding will be carefully investigated. The blood Ionogram will seek the possible presence of an elevation of creatinine and uric acid, particularly encountered in very aggressive lymphomas (tumor lysis syndrome). A liver bioassay will allow suspicion of flooding
6 of this body. Finally, an immuno-electrophoretic examination will seek the presence of a monoclonal blood and urinary component. Some biological elements have a fundamental prognostic significance in non-Hodgkin malignant lymphomas: Serum LDH and beta-2 microglobulin, the elevation of which constitutes significant pejorative prognostic factors; Albumin levels, the significant decrease of which is also a pejorative prognostic factor III-4 Extension to the pith and CNS: The realization of a myelogram and especially a marrow biopsy is indispensable in all malignant lymphomas (60-80% In the low-grade NHL, 25% in the aggressive NHL). The completion of a lumbar puncture is also indispensable in aggressive malignant lymphomas (5% flooding, especially in lymphoblastic and Burkitt). III-5 classification in stages: stage classification is important in determining the prognosis and treatment of lymphomas and the Ann Arbor classification is used:-Stages I correspond to a single ganglionic territory, Whatever the side of the diaphragm. The IE stages which correspond to the damage of a ganglionic territory with a contiguous extra ganglionic attack or an isolated extraganglionnaire attack are distinguished. -Stages II correspond to the attainment of two or more ganglion territories on the same side of the diaphragm; -Stages III correspond to SUS-and sub-diaphragmatic, with the spleen being considered a ganglion when there is a SUS-diaphragmatic (recently, several classifications classify splenic attacks as Stages IV); -Stages IV correspond to a disseminated attack with visceral extension (hepatic, medullary, other organs...). It should be recalled that the extra ganglion of adjacency does not pass into stage 4, but adds the letter E behind the stage (1 E, 2 e) for stages I and II but not stages III. In this staging, letters A or B are associated with the existence or absence of general signs: weight loss, fever, night sweats. The patient is considered a if there are none of these signs, B if there is at least one of the signs of scalability. IV-Prognostic factors: prognostic factors allow for the grouping of patients with nearby prognosis, and to adapt treatments to the risks incurred by the patient. A first prognostic distinction must be made based on histological types of lymphoma (so-called low-grade lymphomas or indolent) and so-called aggressive lymphomas. The
7 Therapeutic indications in these different groups are quite distinct. However, aggressive lymphomas are generally curable, contrary to indolent lymphomas. In these aggressive non-Hodgkin lymphomas, the main prognostic criteria are:-The rate of LDH,-the general condition of the patient (performance scale status or activity index),-a stage III or IV,-an age greater than 60 years-the presence of more than one localizer Extra-ganglionic, these different factors can be grouped in prognostic indexes (presence of 0 to 5 factors) allowing to define groups of patients with homogeneous prognosis. Other prognostic factors identified include:-the existence of anemia-l elevation of Beta2-microglobulin-The decrease in albumin or hemoglobin-the presence of a tumor mass greater than 10 centimetres,-the number of areas Ganglion reached-the presence of a medullary disease. V-Anatomy Pathology of non-Hodgkin lymphoma V-1 classifications The dismemberment of non-Hodgkin lymphomas is complex and has been the subject of many different pathology classifications. The current classifications are based on:-L morphological aspect of the invaded ganglion (diffuse or nodular proliferation)-the cytological aspect of tumor cells (large or small, nucleus, cytoplasm...)-The immunological phenotype of cells Tumours (B or T, associated markers)-additional data from cytogenetic or genetic studies. Immunology (search for intra-cytoplasmic or membrane monoclonal immunoglobulins, search for B-cell or T-cell specific markers) and molecular biology (Study of genes genomic rearrangements of Immunoglobulins, or rearrangements of the genes of the T-cell receptor, allowed the different histological forms to be linked to a B or T lymphocyte immunological phenotype. This distinguishes several categories of non-Hodgkin lymphomas that have recently been characterized in international classifications such as the REAL (Revised European American Lymphoma classification) or who. Each entity is often associated with a particular clinical presentation and some scalability (indolent lymphomas, aggressive lymphomas...). Many studies are currently aimed at establishing the interest of markers derived from molecular studies (gene expression profiles, utations, etc.) both to define specific entities with distinct prognosis and to consider Specific targeted therapies.
8 V-2 NHL B-phenotype (85-90% NHL) Aggressive B lymphomas (35-40% NHL):-Diffuse large cell lymphomas (centroblasts, immunoblasts), which are the most common lymphomas (about 35% of the total cases). They may present themselves as ganglion or extra-ganglion diseases, localized or disseminated, in patients of all ages. Specific varieties (Mediastinum for example) are described. -Burkitt-type lymphomas, with small non-cleaved cells, are rare in adults (about 1% of cases) but very proliferative. Care and treatment are therapeutic emergencies. The cells exhibit a chromosomal translocation still interesting chromosome 8 (oncogene C-MYC) and a region encoding immunoglobulin genes (most often with chromosome 14, t (8; 14) (Q24; q32), where the locus of genes encoding For the heavy chain of the Ig. The treatment relies intensive chemotherapy and about one in two patients will be definitively cured. Follicular lymphomas (20-25% of NHL) proliferation invades the ganglion in the form of tumour follicles. The presentation is essentially ganglionic, and the evolution is gladly slow for several years. The initial prognosis is favorable, but the definitive cure is difficult to obtain. They are characterized by the translocation T (14; 18) (Q32; q21) which rearranges the BCL-2 gene (inhibits apoptosis) near the immunoglobulin genes. Other small cell B lymphomas (20 to 30% of NHL) Malt lymphomas (5-10%): These are small B-cell lymphomas developing at the expense of mucosal lymphoid tissue (malt in English). They preferentially touch the digestive tract (stomach), lung, thyroid, orbit, skin,... (almost all epithelium have associated lymphoid tissue). With regard to stomach MALT lymphomas, chronic infection with Helicobacter Pylori is associated with more than 90% of cases and seems to play a decisive role in the genesis of lymphoma. The various treatments (chemotherapy, sometimes surgery or radiotherapy) often allow to control the disease for many years, even if relapses in the same organ (or at a distance in another organ with MALT) occur quite Frequently but remain available for treatment. -lymphocytic lymphomas (5-10%) correspond to the presence of small lymphocytes in the lymph nodes, similar to those found in the blood and marrow of subjects suffering from chronic lymphoid leukemia. It is the exclusive or predominant ganglionic form (possible blood and splenic) of this disease. The lymphomas of the marginal zone and the cell lymphomas of the mantle correspond to other ganglion entities, sometimes with blood and medullary, splenic. The prognosis of the mantle's lymphomas is particularly bleak despite a often unsettling initial presentation. The treatment modalities of these last three entities are to be adapted to the prognostic factors present in the diagnosis.
9 v - 3 lymphomas of phenotype T (about 10%) classic forms have a basically ganglionic presentation with clinical presentation often noisy (fever, alteration of the general State, expressions of autoimmune allure in the) angio-immunoblastique Lymphadenopathy). The prognosis is more severe (quick scalability, relapse, etc...). It s is also aggressive lymphomas, but patients can be cured in. Rarer forms large cell anaplastic lymphomas, causing problems of differential diagnosis with undifferentiated carcinomas. These lymphomas express leukocyte antigen CD30 (Ki-1). They s often accompany extra-ganglionnaires locations (skin, bone, lung...). Their prognosis is more favorable. T epidermotropes lymphomas, where lymphocyte cells have a very particular tropism for the skin: the most common is the mycosis fungoides; Leukemic form rarely (diagnosis, but more frequent during the evolution) named after Lymphoma Sézary cells. Many other rare lymphomas T entities are described (hepato-splenic, nasal Lymphoma, lymphoma of the small intestine associated with celiac...). VI - THE TREATMENT OF LYMPHOMAS. In malignant lymphomas as in the whole of malignant hematologic diseases, the principle of treatment is based on the start-up of a therapy to quickly obtain complete remission, only guarantee to be able to possibly get a prolonged survival (indolent lymphomas) or a cure. Therapeutics are different in Hodgkin's disease and non-hodgkiniens lymphoma. VI - 1 indolent or low-grade Lymphoma prognosis of low grade lymphomas is quite special: long survival of patients with this pathology, with 60 to 70 percent of survivors to 10 years, but lack of plateau (reflecting a healing) in the curves of survival. This reflects the inexorable progression of almost constant pathology in these patients. The schematic therapeutic indications are then:-localized exclusive radiotherapy or l abstention with close supervision in the forms very localized (stage I and II), less used to the benefit of the following options, but an option in some cases. -therapeutic abstention or the such (Cytoxan, Fludarabine, clinical), in forms without adverse prognostic factors, most recently only used monoclonal antibodies; -polychimiotherapies, always associated antibodies monoclonal anti-cd20, type CVP (Cytoxan, Vincristine, Prednisone), mini CHOP (CVP with addition of ADRIAMYCIN at low dose) or CHOP, or bendamustine for patients with a high mass tumor. These treatments have recently made evidence of a significant improvement in the hope of
10 life of patients: d a median survival of 10 to 12 years ago, there is today in a median survival of 16 to 20 years. -treatment maintenance by monoclonal antibody administered every 2 or 3 months for 2 years in the answering patient. Therapeutic progress remains to be done in this disease primarily in two situations:-when it affects young subjects but with unfavorable prognostic factors: aggravations with autograft have been proposed and present a some interest, particularly in relapse; l Allograft could bring a definitive remission but is rarely achieved because of its toxicity, while the life expectancy of these patients to the diagnosis is good; -for their evolution, these lymphomas low "de grade"may present a "transformation histologique", IE progress in the form of a much more aggressive histology (increase in the number of large cells, transition to a character diffuse). Clinically, evolution is quickly unfavorable despite more intensive chemotherapy. VI - 2 - aggressive lymphomas this pathology has seen his prognosis improved considerably in the last 10 years by the use of intensive chemotherapy, then recently with the antibody monoclonal anti-CD20. Current protocols include always an anthracycline (ADRIAMYCIN or derived) and an alkylating (cyclophosphamide-Endoxan), usually an alkaloid (vincristine or vindesine) and corticosteroids, with more or less, Bleomycin and Methotrexate,... Obtaining complete remission must be fast, and other products can be used in consolidation. The current duration of treatment generally does not exceed 6 months. During and at the end of the treatment, the 18FDG PET-scan is used to evaluate the quality of the response. This is called "metabolic response", which associated morphological criteria classic, is a key factor in the prognosis. In the B Lymphoma, immunotherapy with antibodies monoclonal anti-cd20 is systematically associated with currently (see below). These treatments are important, including hematologic toxicity requiring their realization in services accustomed to hematologic resuscitation (including antibiotics). In the forms meningeal high-risk chemotherapy intra-thecale by repeated injections of Methotrexate is also Assistant. In very proliferative forms (high grade, cuts... NHL), chemotherapy should be started very quickly by combining a hyperhydration with alkalization and inhibitors of the synthesis of the uric acid (allopurinol) and or urico-eliminators (rasburicase to prevent and treat Lysis syndrome: elevation of uric acid, creatinine, phosphorous, which can lead to serious kidney failure). Chemotherapy protocols allow to obtain a complete remission rate between 70 and 80 percent and a nearby 5-year survival rate of 65% (75-85% for young people; ~ 50-60% after 60 years). Therapeutic progress are still to be done in patients with unfavorable prognostic factors: age, large tumor mass. In young subjects to bad
11 prognosis, conducting intensive chemotherapy followed for consolidation by autograft is an interesting approach, when the first complete remission or relapses. The pursuit of the improvement of the results 10 years requires the treatment of these patients by protocols adapted to the stage and the prognosis, in multicenter cooperative trials. VI-3 new tools the aggravations with hematopoietic stem cell transplant: patients young n having not achieved remission full or in relapse only used monoclonal antibodies (direct cytotoxic effect or mediated by the) immune cells or supplement), coupled to toxins (immunoconjugues) or radiolabelled (couples to an isotope). etc new families of drugs are under study: inhibitors of kinases, immunomodulators, these new strategies must be assessed to establish their benefit (survival, cost, quality of life, etc...). VI - 4 the contribution of antibodies monoclonal anti-b in the early 2000s since 5 years, the use of a monoclonal antibody directed against the Antigen CD20, which is expressed on the surface of normal and Lymphoma B cells was evaluated in several trials Therapeutics in B lymphomas. This antibody (rituximab or Mabthera) produced by biotechnology is a hybrid molecule of the d constant portions a human IgG3 and variables/hypervariables portions of immunoglobulins obtained in mice. After recognition of the target (Ag CD20 on cells), it can activate different parts of the immune system (in particular the cytotoxicity dependent d antibodies and Lysis mediated by the complement cascade). It can also induce cell death of tumor B cells by apoptosis, with a probable synergy with conventional chemotherapy molecules directly. Side effects are mainly represented by reactions (fever, chills, sometimes bronchospasm or other allergic reactions) during the first infusion, reactions that can be controlled by the rate of reduction administration of infusion and corticosteroids. These effects are increased and can be dangerous in patients who have many circulating lymphoma cells. The late severe immunological mechanism as well as skin reactions have been reported. Those moderate effects allow the simple association of these treatments to conventional chemotherapy. Used alone (monotherapy), this s antibody proved active in indolent lymphomas where it is part of therapeutic weapons at certain stages of the disease. Maintenance treatments are in the study.
12. in combination with chemotherapy in the aggressive lymphomas, rituximab increases the percentage of full response at the end of the treatment and especially the overall survival of patients with 10 to 20% of additional patients cured (according to the age and the) severity of the disease). In indolent Lymphoma, rituximab combined with chemotherapy also increases the quality of the response to treatment and disease duration, but it seems also bring benefits in terms of lengthening of survival, even if it is not possible currently d said that it will cure some patients given the chronic nature of this disease. This type of treatment has opened a new era in the management of lymphomas and, more generally, in the use of monoclonal antibodies in cancer. New similar molecules are under development. Should be precisely assessed if new benefits can be obtained in the short and long term in this condition through these new therapeutic tools.
13 POINTS FORTS on 1 LYMPHOMAS. Cancer whose frequency increases but no known cause for this increase. 2. the clinical signs of calls are very varied: adenopathy isolated or multiple, superficial or deep (symptomatology aspecific = more late diagnosis), associated or not with General signs. clinical symptoms in connection with leaking d a body (skin, lung, stomach, brain). 3. the diagnosis is based on a tumor biopsy (ganglion or affected organ), which must be done in the best possible conditions because additional tests (Immunology, molecular biology and cytogenetics) must be completed for specify this diagnosis. 4. There are many pathological forms of lymphoma and very schematically distinguished: a. the aggressive so-called B (diffuse large B cell Lymphoma) lymphomas, b. Lymphoma B say "indolent" (of which the most common is the Lymphoma Follicular), c. T lymphomas, most aggressive. 5. the balance sheet expansion will comprise at least a careful clinical examination, an extended CT examination, a biological assessment with complete blood count, renal and hepatic functions, dosage of LDH and b2-microglobulin, a biopsy of the spinal cord and a lumbar puncture (aggressive lymphomas and T lymphomas). 6. the prognosis depends on several clinical and biological criteria including the classification stage d Ann Arbor, the age, the rate of serum LDH, the general State (index of activity) and with extra-ganglionnaires sites. In indolent B lymphomas, d ganglion areas with noimbre, B, d symptoms anemia and beta2-microglobulin are also involved. 7. the treatment is chemotherapy combined with l (antibody anti-cd20) immunotherapy for lymphomas B. Treatments should allow to get a complete answer (assessment by pet 18 - FDG) which: a. may lead to a prolonged remissions (synonyms for healing) in the aggressive lymphomas B or T (the proportion of patients in 5 years survivors Depending on the original prognostic criteria), (b) or goal d will lengthen the range without disease and survival time in indolent lymphomas when treatment is indicated as a result of the tumor mass or symptoms associated with the disease (but the answers durable with healing are very rare in these cases). References to go further: monograph of the journal of the practitioner January 2010 no hodgkiniens (work directed by C Gisselbrecht) lymphomas, Collection CME from the journal of Hematology, John Libbey Eurotext 2008 G rooms _ October 2011
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