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Lymphoma and Multiple Myeloma

Lymphoma and Multiple Myeloma. Terry Hayes, M.D., Ph.D. Topics to be Covered. Non-Hodgkin’s Lymphoma Hodgkin’s Disease Multiple Myeloma. Lymphoma and Multiple Myeloma 2004 U.S. Predicted Values. Malignancy New Cases Deaths All Cancer s 1,368,030 563,700

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Lymphoma and Multiple Myeloma

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  1. Lymphoma and Multiple Myeloma Terry Hayes, M.D., Ph.D.

  2. Topics to be Covered • Non-Hodgkin’s Lymphoma • Hodgkin’s Disease • Multiple Myeloma

  3. Lymphoma and Multiple Myeloma2004 U.S. Predicted Values Malignancy New Cases Deaths All Cancer s 1,368,030 563,700 Non-Hodgkin’s Lymphoma 54,370 19,410 Hodgkin’s Disease 7,880 1,320 Multiple Myeloma 15,270 11,070 CA Cancer J Clin 2004; 54:8-29

  4. Non-Hodgkin’s Lymphoma

  5. Jacqueline Kennedy Onassis Former First Lady

  6. King Hussein of Jordan

  7. “Mr. T” (Lawrence Tureaud) Television star, The A-Team. Sylvester Stallone's adversary in "Rocky III.”

  8. Non-Hodgkin’s Lymphoma • 6th most common cause of cancer death in United States. • Increasing in incidence and mortality. • Since 1970, the incidence of lymphoma has almost doubled.

  9. Overview • The types of non- Hodgkin’s lymphoma reflect the developmental stages of lymphocytes. • Each type of lymphoma can be viewed as a lymphocyte arrested at a certain stage of development and transformed into a malignant cell. • 85% B cell origin, the rest T or null cell.

  10. Precursor B Cell Leukemias CLL, B Cell Lymphomas Waldenström’s, Myeloma B CELL DIFFERENTIATION Ig sIgM,G,A sIgM,D sIgM Cell Surface Markers CD19 CALLA (CD10) CD20 CD38

  11. A B C D E small cleaved large cleaved MATURATION IN LYMPHOID FOLLICLE immunoblast B lympho- cyte F G small non- cleaved large non- cleaved H T lymphocyte plasma cell small lymphocyte

  12. Follicular lymphoma Burkitt’s lymphoma Mantle zone lymphoma Sézary syndrome Mycosis fungoides Peripheral T cell lymphoma Chronic lymphocytic leukemia Small lymphocytic lymphoma Waldenström’s macroglobulinemia

  13. Types of Lymphoma • Indolent (low grade) • Life expectancy in years, untreated • 85-90% present in Stage III or IV • Incurable • Intermediate • Aggressive (high grade) • Life expectancy in weeks, untreated • Potentially curable

  14. WorkingFormulation Low Grade Small lymphocytic Follicular small cleaved Follicular mixed Rappaport Diffuse well-differentiated lymphocytic (DWDL or WDLL) Nodular poorly differentiated lymphocytic (NPDL) Nodular mixed lymphocytic-histiocytic (NM) Commonly Used Classifications

  15. WorkingFormulation Intermediate Grade Follicular large cell Diffuse small cleaved cell Diffuse mixed Diffuse large cell Rappaport Nodular histiocytic (NH) Diffuse poorly differentiated lymphocytic (DPDL) Diffuse mixed lymphocytic-histiocytic (DM) Diffuse histiocytic (DHL) Commonly Used Classifications

  16. WorkingFormulation High Grade Large cell immunoblastic Lymphoblastic lymphoma Small noncleaved cell • Burkitt’s • Non-Burkitt’s Rappaport Diffuse histiocytic (DHL) Diffuse lymphoblastic Diffuse undifferentiated (DU) Commonly Used Classifications

  17. Median Survival Histology (Years) DWDL 8-12 NPDL 5-8 NM 5-8 NH 1-3 DPDL 2-4 DM 1-3 DHL .5-1.5 DU .6- .7

  18. Not Included inThese Classifications • Mycosis fungoides • Marginal zone B cell lymphoma • MALT lymphoma • Mantle cell lymphoma • Peripheral T cell lymphoma • Angioimmunoblastic lymphoma

  19. The REAL Classification (Revised European-American Lymphoma Classification) September, 1994

  20. REAL Classification • Precursor B-lymphoblastic lymphoma/leukemia • B cell CLL/prolymphocytic leukemia/small lymphocytic leukemia • Lymphoplasmacytoid lymphoma • Mantle cell lymphoma • Follicular center lymphoma, follicular • Follicular center lymphoma, diffuse

  21. REAL Classification • Extranodal marginal zone B cell lymphoma (MALT type) • Nodal marginal zone B cell lymphoma • Splenic marginal zone B-cell lymphoma • Hairy cell leukemia • Plasmacytoma/myeloma

  22. REAL Classification • Diffuse large B cell lymphoma • Primary mediastinal large B cell lymphoma • Burkitt’s lymphoma • High grade B cell lymphoma, Burkitt-like • Precursor T lymphoblastic lymphoma/leukemia • T cell CLL/prolymphocytic leukemia

  23. REAL Classification • Large cell granular lymphocytic leukemia: T cell type, NK cell type • Mycosis fungoides/ Sézary syndrome • Peripheral T cell lymphomas, unspecified • Hepatosplenic g-d T cell lymphoma • Angioimmunoblastic T cell lymphoma

  24. WHO Classification

  25. Types of Non-Hodgkin’s Lymphoma Small lymphocytic Immunoblastic Mantle cell Large Cell

  26. Etiology of NHL • Immune suppression • congenital (Wiskott-Aldrich) • organ transplant (cyclosporine) • AIDS • increasing age • DNA repair defects • ataxia telangiectasia • xeroderma pigmentosum

  27. Etiology of NHL • Chronic inflammation and antigenic stimulation • Helicobacter pylori inflammation, stomach • Chlamydia psittaci inflammation, ocular adnexal tissues • Sjögren’s syndrome • Viral causes • EBV and Burkitt’s lymphoma • HTLV-I and T cell leukemia-lymphoma • HTLV-V and cutaneous T cell lymphoma • Hepatitis C

  28. Epidemiology • Can occur at any age • Overall incidence, and incidence of subtypes, varies with location: • Burkitt’s in tropical Africa • IPSID in Middle East • Adult T cell leukemia-lymphoma in Japan and Caribbean

  29. Epidemiology • Indolent lymphomas are rare in young people and increase in incidence with age. • Large cell lymphoma (DHL) is less age related, and is among most common cancers affecting the young. • Burkitt’s and lymphoblastic lymphoma are common in adolescents. • AIDS patients develop aggressive, high grade lymphomas.

  30. Clinical Features • Lymphadenopathy • Cytopenias • Systemic symptoms • Hepatosplenomegaly • Fever • Night sweats

  31. Clinical Features • Lymphadenopathy may fluctuate or spontaneously remit, especially in low-grade lymphomas. • B symptoms more common in high-grade lymphomas. • Hematogenous spread of disease, with no predictable pattern.

  32. Clinical Features • Classic lymphoma: arises in lymph node or bone marrow. • Extranodal primary more common in high-grade lymphoma. • Waldeyer’s ring involvement frequent in GI lymphomas.

  33. Diagnosis of NHL • Excisional biopsy is preferred to show nodal architecture (follicular vs diffuse). • Immunohistochemistry to confirm cells are lymphoid • LCA (leukocyte common antigen) • Monoclonal staining with Igk or Igl • Flow cytometry: • CD 19, CD20 for B cell lymphomas • CD 3, CD 4, CD8 for T cell lymphomas

  34. Diagnosis of NHL • Chromosome changes • 14;18 translocation in follicular lymphoma • bcl-2 oncogene • t(8;14), t(2;8), t(8;22) in Burkitt’s lymphoma • c-myc oncogene • t(11;14) in mantle cell lymphoma • cyclin D1 gene

  35. Staging Workup • CBC, chemistries, urinalysis • CT scans of chest, abdomen and pelvis • Bone marrow biopsy and aspirate • (Lumbar puncture) • AIDS lymphoma • T cell lymphoblastic lymphoma • High grade lymphoma with positive marrow

  36. Staging laparotomy and lymphangiogram are not indicated in non-Hodgkin’s lymphoma.

  37. Staging: Ann Arbor I. 1 lymph node region or structure II. >1 lymph node region or structure, same side of diaphragm III. Both sides of diaphragm IV. Extranodal sites beyond “E” designation subscripts: A, B, E, S

  38. Treatment Options:Indolent lymphomas WDLL, NPDL • 10-15% in Stage I or II • potentially curable • local radiotherapy • 85-90% Stage III or IV • incurable • treatment does not prolong survival

  39. Reasons to Treat in Advanced Indolent Lymphomas • Constitutional symptoms • Anatomic obstruction • Organ dysfunction • Cosmetic considerations • Painful lymph nodes • Cytopenias

  40. Treatment Options inAdvanced Indolent Lymphomas • Observation only. • Radiotherapy to site of problem. • Systemic chemotherapy • oral agents: chlorambucil and prednisone • IV agents: CHOP, COP, fludarabine, 2-CDA. • Antibody against CD20: Rituxan, Bexxar, Zevalin. • Stem cell or bone marrow transplant.

  41. CHOP Chemotherapy • Cyclophosphamide (Cytoxan) • Hydroxydaunorubicin (Adriamycin) • Oncovin (vincristine) • Prednisone

  42. Treatment Options:Aggressive Lymphomas Aggressive • Diffuse large cell lymphoma, large cell anaplastic lymphoma, peripheral T cell lymphoma. Very Aggressive • Burkitt’s lymphoma and lymphoblastic lymphoma.

  43. Treatment Options for Early Stage Aggressive Lymphomas • Often in Stage I or II • potentially curable • disseminates through bloodstream early • must use systemic chemotherapy • CHOP x 6 cycles • CHOP x 3 cycles followed by radiotherapy

  44. Treatment Options for Advanced Stage Aggressive Lymphomas • Systemic chemotherapy • CHOP (± Rituxan for over 70 age group) • ± Intrathecal chemotherapy • AIDS patients and CNS involvement • ± Radiotherapy • Spinal cord compression, bulky disease

  45. Lymphoblastic Lymphoma • T cell malignancy. • Male adolescents. • Mediastinal mass. • T cell variant of T cell acute lymphoblastic leukemia. • Prognosis improving with intensive ALL regimens.

  46. Burkitt’s Lymphoma • African variety: jaw tumor, strongly linked to Epstein-Barr Virus infection. • In U.S., about 50% EBV infection. • May present as abdominal mass. • Most rapidly growing human tumor. • Typical chromosome abnormality: c-myc oncogene linked to one of the immunoglobulin genes.

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