linfoma no hodgkin

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  1. 1. 371 - UABC FMyP - Hematologa Carrillo Bayln Rafael Enrique
  2. 2. Armitage J, Harris N, Dalla-Favera R. Non-Hodgkin Lympohomas. Lippincot Williams & Wilkins, 2 ed. 2010 Robbins, Cotran, Kummar V, Abbas A, Fausto N, Aster J. Patologa estructural y funcional. Elsevier, 8 ed. 2010 Sans-Sabrafen J, Besses C, Vives JL. Hematologa Clnica. Elsevier, 5 ed. 2006 Non-Hodgkin Lymphoma. National Cancer Institute: US Department of Health and Human Services http://www.cancer.org/cancer/non-hodgkinlymphoma/detailedguide/non-hodgkin-lymphoma- what-is-non-hodgkin-lymphoma Non-Hodgkin Lymphoma: A histopatologic and prognostic evaluation. 2010. Genentech, USA Labardi-Mndez J, Cervera-Ceballos E, Corrales-Alfaro C, Balbuena-Martnez M, Barbosa- Ibarra A, Espinoza-Zamora JR, et al. Onco Gua: Linfoma No Hodgkin. Cancerologia 6 (2011): 139 152
  3. 3. Lymphoma is a cancer of the lymphatic system Sist. Linftico ganglios, vasos y rganos linfticos linfa linfocitos sistema inmune Enfermedades clonales de clulas B, T o NK en varios estadios de diferenciacin Las clulas NK se consideran dentro del mismo grupo con linfocitos T, por sus rasgos inmunofenotpicos y funcionales similares
  4. 4. Non-Hodgkin Lymphoma. National Cancer Institute: US Department of Health and Human Services Diseases need to be defined and named before they can be diagnosed and treated - Disease Definition (Class discovery): is the process of determining what diseases exist and how to define them; developing a classification when applied to a large group of diseases - Disease Diagnosis (Class prediction): is the act of deciding which category of disease a given patient has.
  5. 5. Lymphosarchoma Robert Virchow (1863) lo distingui de la leucemia que el describi (1845) Malignant Lymphoma Theodore Billroth (1871) Controversia para clasificar los NHL basados en: 1) Morfologa 2) Caractersticas clnicas 3) Linaje Celular y diferenciacin 1902, Carl Sternberg and Dorothy Reed described the characteristic binucleate cell that camed to be called. Sternberg-Reed Cell 1908, Sternberg described an aggressive mediastinal tumor in young males (Sternberg Sarchoma) Lymphoblastic Lymphoma
  6. 6. Armitage J, Harris N, Dalla-Favera R. Non-Hodgkin Lympohomas. Lippincot Williams & Wilkins, 2 ed. 2010 Used heterogenously: - Lymphoma - Lymphosarcoma - Follicular lymphoma - Lymphocytoma - Lymphoblastoma - Reticulum cell sarcoma - Hodgkin disease - Morphologic + clinical features
  7. 7. Armitage J, Harris N, Dalla-Favera R. Non-Hodgkin Lympohomas. Lippincot Williams & Wilkins, 2 ed. 2010 Terms such as lymphosaroma or lymphoblastoma were applied to those composed of smaller cells recognized as lymphocytes. Gall: when such variation of opinion exists it seems probable that the individual authorscannot be describing the same tumor 1941, follicular lymphoma or giant follicle lymphoma was recognized by GALL 1958, Burkitt described a tumor of African children, which was rapidly recognized as a new and distinctive type of lymphoma which also occured in the Western countries Led to suspicion that it may be caused by a virus = discovery of the VEB. The term reticulum cell sarcoma was generally applied to large cell neoplasms; this uncertainty about the lineage of large cell neoplasms of lymphoid tissues persisted until later half of the 20th century. In the belief that each neoplasm corresponded to a recognizable normal cell or differentiation stage: 1942: Morphology + different categories = different clinical behavior
  8. 8. Proposed the term nodular to replace follicularwhen describing the pattern of the lymphoma Useful in stratifying patients for treatment and predicting clinical outcome Regression on understanding
  9. 9. Discoveries about inmune system & neoplasms: 1) Potential of lymphocytes: had been thought to be end- stage not proliferating cells in response to mitogens or antigens 2) Existence of distinctive lymphocyte lineages (T/B): w/different funcitons and physiology - Surface antigens exploited to ID lineage 3) Effective therapies for some types of lymphomas
  10. 10. Lymphocyte differentiation (hypothethical scheme) Low-grade malignancy: small cells -cytes High-grade malignancy: -blasts
  11. 11. Armitage J, Harris N, Dalla-Favera R. Non-Hodgkin Lympohomas. Lippincot Williams & Wilkins, 2 ed. 2010 Inmunologically based classification - Required primary division into T and B-cell lineage - It didnt recognize pattern at all - Folicular center cell types = all neoplasms
  12. 12. Non-Hodgkin Lymphoma: A histopatologic and prognostic evaluation. 2010. Genentech, USA
  13. 13. [1] Non-Hodgkin Lymphoma. National Cancer Institute: US Department of Health and Human Services [2] Sans-Sabrafen J, Besses C, Vives JL. Hematologa Clnica. Elsevier, 5 ed. 2006 The most common are diffuse large B-cell lymphoma and follicular lymphoma 2 groups according to the speed of growth: Indolent (low-grade): they grow slowly and cause few symptoms Over time they can become aggresive lymphomas Aggresive (intermediate-grade and high-grade): they grow and spread more quickly; and tend to cause severe symptoms.
  14. 14. Ms frecuentes en adultos que nios e incrementan gradualemente con edad >50a (45-55 edad Dx promedio) NIOS Incidencia es rara, tiene predominio extranodal, el 50-70% presentan inmunofenotipo B, es agresivo y se cura en el 70 al 90% de los casos. ADULTOS Incidencia es alta, tiene predominio nodal, el 70 al 90% corresponden a inmunofenotipo B, curso clinico variable y la tasa de curacin es alrededor del 30% OMS: Tasa de incidencia mundial de LNH en H fue de 5.6/100,000 y la TM 3.2/100,000. En Mujeres la TI y TM fueron < respectivamente: 4.1/100,000 y 2.4/100,000 En MEXICO los datos de Globocan 2002 para en hombres fueron: Hombres TI: 4.5/100,000, TM: 2.1/100,000 Mujeres TI: 3.3/100,000 y TM: 1.6/100,000.
  15. 15. Agentes infecciosos: Virus: VEB (LNH de Burkitt africano, LNH asociado al VIH, linfomas posrtrasplante) HTLVI (leucemia/linfoma T del adulto), etc. Bacterias: Helicobacter pylori (Iinfoma MALT gstrico) B. burgdorferi (LNH MALT cutneo) Chlamydia (LNH MALT de las glndulas lagrimales Alteraciones inmunitarias: Inmunodepresion: VIH, enfermedad injerto contra huesped Enf. Autoinmunes: (tiroiditis de Hashimoto) Exposicin a agentes txicos: Radiaciones ionizantes, herbicidas, pesticidas
  16. 16. [1] Non-Hodgkin Lymphoma. National Cancer Institute: US Department of Health and Human Services Derivadas de infiltracin: 90% adenopatas 50% hepato o esplenomegalia 1/3 MO infiltrada (>en>) 1/3 afectacin extraganglionar - TD (Placas de Peyer) - Orofaringe (Anillo de Waldeyer) - Mediastino - SNC - Piel Sntomas B o constitucionales: 60% Estadios avanzados 1/3 sntomas es (+) 1) Fiebre inexplicada >38 C 2) Sudoracin nocturna 3) Prdida de peso injustificada de >10% en 6m precedentes Ganglios inflamados, s/dolor: Cuello, axilas, inguinales Prdida de peso inexplicable Fiebre Sudoracin nocturba Tos, disnea, dolor torcico Astenia Dolor, inflamacin o sentimiento de plenitud abdominal Infecciones o algun problema alterno puede ser la causa de estos sntomas
  17. 17. Debe realizarse en tejido ganglionar o extraganglionar obtenido preferentemente por biopsia escisional. Inmunohistoquimica minima obligatoria: CD45, CD20 y CD3. Deber complementarse con la sospecha diagnstica.
  18. 18. Non-Hodgkin Lymphoma: A histopatologic and prognostic evaluation. 2010. Genentech, USA
  19. 19. Non-Hodgkin Lymphoma: A histopatologic and prognostic evaluation. 2010. Genentech, USA
  20. 20. CD10 +/-, CD20 -/+, CD79a -/+: bcl-2 +/-, Tdt +
  21. 21. Neoplasia de celulas precursoras de linaje B o T 75% nios 25 Blastos=leucemia Pueden afectar a la MO, sangre, sitios nodales y extranodales. Se diferencia de LAL por su presentacin clinica con masa tumoral y la presencia o no de infiltracin en MO de celulas con cromatina madura. Diagnostico El abordaje por inmunofenotipo, cariotipo y biologia molecular en la MO LAL Inmunohistoquimica en tejidos: CD19, CD79a, CD22, CD10, bcl2 y TdT. Biopsia de ganglio, tejido o aspirado de MO Origen en celulas T: Tumor mediastinal, adenomegalias y hepatoesplenomegalia y puede infiltrar el SNCy testiculos.
  22. 22. al de LAL Se agrega Rituximab 375 mg/m2 en pacientes que expresan: CD20 por inmunohistoqumica o CD20 en ms del 10% por inmunofenotipo
  23. 23. 48% del total de linfomas en Mexico. Dx en tejido ganglionar/extraganglionar por biopsia escicional preferentemente Inmunohistoquimica minima obligatoria: CD45, CD20 y CD3 Complementada con BCL-2, BCL-6, MUM-1, CD-10, CD-30 y ALK. La valoracin del riesgo se calcular de acuerdo al (IPI) e IPI ajustado a la edad
  24. 24. Infiltracin a SNC, iniciar Tx intratecal: Metotrexate 12 mg Citarabina 40 mg Dexametasona 4 mg 2 x sem hasta obtener 3 LCR (-) Evaluar RT o dosis altas de metotrexate.
  25. 25. Neoplasia de celulas B del centro germinal (centrocitos y centroblastos) con un patrn de crecimiento folicular y bajo grado de agresividad. 20% de todos los LNH. Afecta predominantemente a adultos, 60-70 anos Manifestaciones clinicas Enfermedad en estadios clinicos avanzados y con infiltracin a MO en 40 a 70%; s/MsCs Predominio nodal Diagnostico Especificar el grado histolgico de acuerdo al # de centroblastos por campo de alto poder (grado 1-2; 15 centroblastos, grado 3 >15 centroblastos) El linfoma folicular grado 3b (75% de patron difuso) se considera un linfoma agresivo y asi debe ser tratado Inmunohistoquimica incluya al menos: CD20, BCL2, CD10.
  26. 26. Mantenimiento Rituximab 375 mg/m2 cada 3 meses durante 2 anos.
  27. 27. Linfoma de celulas B de crecimiento rapido Con fc se presenta con infiltracin extranodal o leucemia aguda. Clulas mo