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MALIGNANT LYMPHOMAS

MALIGNANT LYMPHOMAS. Dr. Manjit Singh Saren MBBS, MCPS, DTM&H, DCP, D. Path MAHSA University College. LYMPHOID NEOPLASIA Malignant monoclonal proliferation of lymphoid cells. Involves lympho-reticular sites: Liver, spleen, GIT and bone marrow.

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MALIGNANT LYMPHOMAS

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  1. MALIGNANT LYMPHOMAS Dr. Manjit Singh Saren MBBS, MCPS, DTM&H, DCP, D. Path MAHSA University College

  2. LYMPHOID NEOPLASIA Malignant monoclonal proliferation of lymphoid cells. Involves lympho-reticular sites: Liver, spleen, GIT and bone marrow. Involvement of bone marrow causes leukemia Conversely leukemia to lymphoma

  3. LYMPHOMA CLASSIFICATION B-Cell Lymphoma: Derived from follicular centre or post follicular centre. T-Cell Lymphoma: Thymic in origin Migration of lymphocytes to thymus

  4. LYMPHOID MALINANCIES FREQUENCY: Non Hodgkin’s Lymphoma 62 % Plasma Cell Disorders 15 % Hodgkin’s Lymphoma 8 % Chronic Lymphocytic Leukemia 9% Acute Lymphocytic Leukemia 4%

  5. HODGKIN’S DISEASEHODGKIN’S LYMPHOMA

  6. RYE’S CLASSIFICATIONPROGNOSIS i) Nodular sclerosis 70% Very good ii) Lymphocyte rich 5% Excellent iii) Mixed cellularity 22% Good iv) Lymphocyte depletion 1% Poor

  7. HODGKIN’S LYMPHOMA Incidence: 8% of lymphoid malignancies. Features: Distinct group of neoplasm. Affects contiguous nodes or extra-nodal sites Age: Bimodal: 15-35 yrs : 50 yrs +

  8. HODGKIN’S LYMPHOMA Etiology: i) Hereditary: 99 X in identical twins ii) Familial incidence: (HLA histo-compatibility) iii) Virus: Ebstein- Bar virus genome EB Virus: 70% cases of mixed cellularity

  9. HODGKIN’S LYMPHOMA Micro: “Abnormal giant cells with multi-lobed nuclei, prominent acidophilic nucleoli giving a Mirror Image or OWL’S EYE appearance” Known as: Dorothy-Reed-Sternberg cells

  10. REED STERNBERG CELL

  11. H.D. PATHOGENESIS: Reed Sternberg cells secrete cytokines: i. IL-8 (Attracting eosinophils) ii. IL-5 (growth factor for eosinophils) iii. IL-13 (stimulation of R-S cells) iv. Growth factor-B (fibro-genesis)

  12. HODGKIN’S LYMPHOMA Clinical Features: 1. Cervical and mediastinal lymphadenopathy Painless, mobile and firm. 2. Hepato-splenomegaly 3. Fever 4. Night sweats. 4. Weight loss, fatigue and malaise

  13. Micro: 1.Dorothy-Reed Sternberg Cells 2.’Pop corn’ cells (Lympho-Histiocytes) 3.Lacunar cells 4.Eosinophils

  14. LABORATORY DIAGNOSIS • Normocytic hypochromic anemia. • Eosinophilia • HISTOPATHOLOGICAL • i. Haematoxylin and Eosin stains • ii. Immuno-histochemical stains: • CD 20 for B Cells • CD 15 and CD 30 for Hodgkin’s Cells • iii. X Ray and CT scan Chest and Abdomen

  15. ANN ARBOR STAGING I. Single lymph node group or extra lymphatic organ or siteII. Multiple lymph nodes ( same side of diaphragm)III. L. Nodes on both sides of diaphragmIV. Multiple extra nodal sites and Marrow Bulk>10cm: Extra nodal extension

  16. PROGNOSIS 5 year survival: Ann Arbor Staging. I, II, III, IV Stage I and II: 100 % Advanced stage: 50 % Lymphocyte predominant: Best prognosis Lymphocyte depleted: Poor prognosis. Treatment: Aggressive chemo-radiotherapy

  17. NON- HODGKIN’S LYMPHOMASDr. Manjit Singh SarenMAHSA UniversityCollege

  18. Subcapsular Sinus • B cells • Cortex Medulla • T cells Lymphoid Follicle

  19. B cells Germinal Centre Lymphoid Follicle

  20. INTRODUCTION NHL: Rising incidence. Group of lympho-proliferative disorder. Affects B and T and Natural Killer lymphocytes High mortality rate.

  21. Incidence: B-CELL lymphomas: 80-90%T-CELLlymphomas:15-20%NK Lymphomas: Rare In Malaysia: NHL 3rd commonest cancer

  22. WHO CLASSIFICATION 1. B-CELL LYMPHOMA2. T-CELL LYMPHOMA 3. NATURAL KILLER CELL LYMPHOMA (NK)

  23. 1. B CELL: NON HODGKIN’S LYMPHOMA i ) MALT Lymphoma 6%* ii) Follicular Lymphoma 22%* iii) Diffuse large cell Lymphoma 31 %* iv) Burkitt’s Lymphoma 2.5%*

  24. 2. T CELL: NON HODGKIN’S LYMPHOMA 1. Extra nodal NK cell lymphoma (Nasal Type)*2. Mycosis Fungoides/Sezary syndrome*

  25. B and T CELL LYMPHOMA1. IMMATURE CELL LYMPHOMA2. MATURE CELL LYMPHOMA

  26. CLINICAL MANIFESTATIONS: 1. Lymphadenopathy 2. Extra-nodal involvement: i. Splenomegaly ii. Anterior mediastinal mass (with Supra-Vena Cava obstruction) iii. Bone Marrow iv. Central Nervous System

  27. IMMATURE B CELL and T CELL: • LYMPHOBLASTIC LYMPHOMA • Age: children/young adults • Cells: Immature lymphocytes • i)B Cells: > Lymphoblasts • ii) T Cells: > Lymphoblasts • Transforms: Acute Lymphoblastic Leukemia

  28. IMMATUREB CELL: LYMPHOBLASTIC LYMPHOMA: Clinical Presentation:i) Lymphadenopathy.ii) Hepato-splenomegaly.iii) CNS and cutaneous infiltration.iv) Blood and Marrow involvement.Complication: LEUKEMIA

  29. Cervical lymphadenopathy

  30. IMMATUREB CELLLYMPHOMA: LYMPHOBLASTIC LYMPHOMA INVESTIGATIONS:CBC:Anaemia, Thrombocytopenia and NeutropeniaPBF: Lymphoblasts and smudge cells.Bone Marrow:Malignant undifferentiated cellsMegakaryocytes: Reduced

  31. INVESTIGATIONS: IMMATURE B CELL LYMPHOMA Stains : i) Periodic Acid Schiff: + ve (PAS) ii) Myelo-peroxidase: - ve Immunophenotypes:B Cells: CD 20 +ve T Cells: CD 3 and 5 +veCytogenetics: t(9;20) Philadelphia +ve A.L.L.

  32. MATUREB CELL LYMPHOMAS SMALL CELL LYMPHOMAAssociated with:Breakdown immune regulations Hypogamma-globulinemia AUTO-ANTIBODIES: 1. AIHA 2. Thrombocytopenia Complication: Large B-Cell Lymphoma

  33. NON HODGKIN’S T CELL LYMPHOMA Clinical Presentation: Lymphadenopathy Hepatosplenomegaly Leukemia (marrow invasion) Immuno-phenotype: CD 3 and 5+ve CD 20 -ve

  34. LABORATORY DIAGNOSIS. SMALL CELL LYMPHOMAi) Anaemia (20% AIHA)ii) 90% mature lymphocytes + ‘smudge cells’iii) Platelets: reducedL.N. Biopsy: Diffuse replacement by small mature B lymphocytes

  35. 1. FOLLICULAR B CELL LYMPHOMAAge: Elderly Clinical Presentation:Lymphadenopathy Micro: Grades: 1,2,3. Loss of normal architecture. Replaced by nodal pattern No mitosis or apoptosis

  36. FOLLICULAR LYMPHOMA ETIOLOGY: B Cl 2 oncogene Immune-phenotype: CD 20 and B Cl 2 +ve 40% > Diffuse large B cell. Prognosis: BAD

  37. 2. MALT B-CELLLYMPHOMA Extra Nodal Lymphoma MALTOMA= Commonest gastric lymphoma Etiology: Helicobacter Pylori Inactivation: p53 Tumour suppressor gene. Micro: Diffuse sheets of small lymphocytes Immuno-phenotype: CD: 20+ve CD: 3 and 5-ve

  38. 3.DIFFUSE LARGE B CELL LYMPHOMA Most common. Age: Elderly Clinical Presentation:i. Lymphadenopathyii. Extra nodal mass: Bone marrow or GIT, Primary CNS mass Mediastinal mass with effusion Abdominal mass

  39. DIFFUSE LARGEB CELLLYMPHOMA Predisposing Factors: 1) Congenital & acquired immunodeficiency 2) EBV in immuno-deficiency states 3) Human Herpes Virus Type 8 (HHV-8) 4) Tumour suppression p53 gene inactivation

  40. DIFFUSE LARGE B CELL LYMPHOMA Micro: Diffuse nodal effacement Large cells with large nuclei and nucleoli. Immuno-phenotype:CD 20 and CD10 +ve CD 3 and 5 -ve Prognosis: Poor Very aggressive

  41. BURKITT’S LYMPHOMA

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