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Lymphomas

Lymphomas. WHO Lymphoid Neoplasms. B cell neoplasms T/NK cell neoplasms Hodgkin lymphoma (disease). 2008 Estimated US Cancer Cases*. Men 720,280. Women 679,510. Prostate 33% Lung & bronchus 13% Colon & rectum 10% Urinary bladder 6% Melanoma of skin 5%

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Lymphomas

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  1. Lymphomas

  2. WHO Lymphoid Neoplasms • B cell neoplasms • T/NK cell neoplasms • Hodgkin lymphoma (disease)

  3. 2008 Estimated US Cancer Cases* Men720,280 Women679,510 Prostate 33% Lung & bronchus 13% Colon & rectum 10% Urinary bladder 6% Melanoma of skin 5% Non-Hodgkin 4% lymphoma Kidney 3% Oral cavity 3% Leukemia 3% Pancreas 2% All Other Sites 18% 31% Breast 12% Lung & bronchus 11% Colon & rectum 6% Uterine corpus 4% Non-Hodgkin lymphoma 4% Melanoma of skin 3% Thyroid 3% Ovary 2% Urinary bladder 2% Pancreas 22% All Other Sites *Excludes basal and squamous cell skin cancers and in situ carcinomas except urinary bladder. Source: American Cancer Society, 2008.

  4. Lymphoma • Clonal malignant disorders that are derived from lymphoid cells: either precursor or mature T-cell or B-cell • Majority are of B- cell origin • Divided into 2 main types : 1. Hodgkin’s lymphoma 2. Non - Hodgkin’s lymphoma

  5. Hodgkin’s Disease • Histologically & clinically a distinct malignant disease • Predominantly, B-cell disease • Course of the disease is variable, but the prognosis has improved with modern treatment

  6. B cell malignancies Lymph node, lymph, blood, bone marrow Lymph node, lymph, blood, bone marrow Bone marrow Bone marrow Progressive B lymphocyte maturation Lymphoid stem cell Mature B cell Plasma cell Maturing B cell many stages Pre-B acute lympho- blastic leukemia B cell lymphoma Chronic lympho- cytic leukemia Multiple myeloma

  7. Clinical features • Bimodal agedistribution : • young adults ( 20-30 yrs) & elderly (> 50yrs) may occur at any age • M > F • Lymphadenopathy: • most often cervical region • asymmetrical, discrete • painless, non-tender • elastic character on palpation ( rubbery) • not adherent to skin

  8. Constitutional symptoms ( B symptoms ) Night sweats, sustained fever > 38 degree celsius, loss of weight >10% of body weight in 6 mo Fever sometimes cyclical (‘Pel-Ebstein fever’) Pain at the site of disease after drinking alcohol Pallor Pruritis Symptoms of Bulky (>10 cm) disease

  9. Lymph node biopsy : Malignant REED-STERNBERG ( RS) Cell: Bi-nucleate cell with a prominent nucleolus. Derived from B cell, at an early stage of differentiation Reactive background of eosinophils, lymphocytes, plasma cells Fibrous tissue

  10. REED-STERNBERG ( RS ) Cell

  11. Hodgkin’s Disease/LymphomaIn the Beginning Accounts for ~ 30% of all malignant lymphomas Composed of two different disease entities: Lymphocyte-predominant Hodgkin’s (LPHD), making up ~ 5% of cases Classical HD, representing ~ 95% of all HDs. A common factor of both HD types is that neoplastic cells constitute only a small minority of the cells in the affected tissue, often corresponding to < 2% of the total tumor

  12. Fatal disease with 90% of untreated patients dying within 2 to 3 years With chemotherapy, >80% of patients suffering from HD are cured. Pathogenesis of HD is still largely unknown. HD nearly always arises and disseminates in lymph nodes Hodgkin’s Disease/LymphomaIn the Beginning

  13. Hodgkin’s Disease/LymphomaClinical Presentation Stage Definition I Involvement of a single lymph node region (I) or of a single extralymphatic organ or site (IE) II Involvement of two or more lymph node regions on the same side of the diaphragm (II) or localized involvement of an extralymphatic organ or site and one or more lymph node regions on the same side of the diaphragm (IIE) III Involvement of lymph node regions on both sides of the diaphragm (III) which may be accompanied by involvement of the spleen (IIIS) or by localized involvement of an extralymphatic organ or site (IIIE) or both (IIISE) IV Diffuse or disseminated involvement of one or more extra lymphatic organs or tissues with or without associated lymph node involvement B symptoms: fever > 38ºC for three consecutive days, drenching night sweats or unexplained loss 10% or more of weight the preceding 6 months

  14. Non Hodgkin’s lymphoma • Incidence is increasing • NHL>HD • Median age of presentation is 65-70 yrs • M>F • More often clinically disseminated at diagnosis • B-cell-70% ; T-cell-30%

  15. Staging • Stage I : Involvement of single LN region (I) or extra lymphatic site (IAE ) • Stage II :Two or more LN regions involved (II) or an extra lymphatic site and lymph node regions on the same side of diaphragm • Stage III : Involvement of lymph node regions on both sides of diaphragm, with (IIIE) or without (III) localized extra lymphatic involvement or involvement of the spleen (IIS) or both (IISE) • Stage IV :Involvement outside LN areas (Liver, bone marrow) A : Absence of ‘B’ symptoms B : B symptoms present

  16. Clinical features • Widely disseminated at presentation • Nodal involvement: Painless lymphadenopathy, often cervical region is the most common presentation • Hepatosplenomegaly • Extranodal : • Intestinal lymphoma( abdominal pain, anemia, dysphagia); CNS( headache, cranial nerve palsies, spinal cord compression) ; Skin, Testis; Thyroid; LungBone marrow(low grade): Pancytopenia

  17. Systemic symptoms • Sweating, weight loss, itching • Metabolic complications:hyperuricemia, hypercalcemia, renal failure • Compression syndrome: • Gut obstruction • Ascites

  18. Diagnosis and staging • Similar to HD plus, • Bone marrow aspirate & trephine • Immunophenotyping : Monoclonal antibodies directed against specific lymphocyte associated antigens B cell antigens ( CD 19, 20, 22); T cell antigens ( CD 2, 3, 5 & 7) • Immunoglobulin determination: IgG / IgMpraprotein marker • HIV • CT / MRI

  19. Non-Hodgkin’s LymphomaRappaport Classification • Small cell, follicular • Small cell, diffuse • Large cell, follicular • Large cell, diffuse

  20. Non-Hodgkin’s LymphomaRappaport Classification Nodular (follicular) Diffuse Indolent Aggressive Small cell Large cell

  21. Classification Low grade Proliferation: Low Course: Indolent Symptoms: -ve Treatment: Not curable High grade High Rapid, fatal(un-Rx) +ve Potentially Curable Staging Similar to HD

  22. Etiology • Cannot be attributed a single cause • Chromosomal translocations: t (14, 18) • Infection: • Virus:EBV, HTLV,HHV-8, HIV • Bacteria: H.Pylori - Gastric lymphoma • Immunology: • Congenital immunodeficiency, • Immunocompromised patients - HIV, organ transplantation

  23. Non-Hodgkin’s LymphomaImmunophenotyping • Immunohistochemistry • Immunofluorescence • Flow cytometry • Identification of CD’s (cluster determinants) • CD5 = T cell type • CD20 = B cell type

  24. Non-Hodgkin’s LymphomaBurkitt’s NHL • Very Aggressive • Curable with standard-dose therapy but requires very extensive chemotherapy protocol • Translocation t(8,14) • Specific Hematopathology Finding • Starry, Starry Night

  25. Burkitt’s LymhomaStarry, Starry Night

  26. Burkitt’s Lymphoma –Background * Burkitt's lymphoma (BL) is a tumor which was first described in 1958 by Denis Burkitt, a surgeon working in Africa. It is a solid tumor of B Lymphocytes which form part of the white cell population in the blood and lymph glands. It is one form of non-Hodgkin's lymphoma.

  27. Burkitt’s Lymphoma -Karyotype: * Here is an actual karyotype (courtesy of Janet Finan and C. M. Croce) of a cell from the tumor of a patient with Burkitt's lymphoma. The long (q) arm of the resulting chromosome 8 is shorter (8q-) than its normal homologue; the long arm of translocated chromosome 14 longer (14q+).

  28. Burkitt’s Lymphoma -Incidence • The incidence of Burkitt's lymphoma shows great geographical variation. It is the most common childhood tumor in equatorial Africa but is very rare in children in Western countries. Recently Burkitt's lymphoma has been diagnosed in around 2% of AIDS patients. • Males are three times more likely to suffer from othis disorder as opposed to females. • Burkitt’s is a rare form of cancer and there are only about 100 new cases each year in the United States.  

  29. Burkitt’s Lymphoma -Causes * In almost all cases of African Burkitt's lymphoma the cells carry a virus known as Epstein-Barr virus (EBV) named after the two British scientists who discovered it.  * This virus is very common and over 90% of adults throughout the world have evidence of past infection. Clearly not everyone develops the tumour and so EBV alone does not cause Burkitt's lymphoma. It must be only one of the factors necessary for the disease to develop. * Other possible contributory factors are chronic malaria infection in Africa and infection with the AIDS virus (human immunodeficiency virus - HIV) in Western countries.

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