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Lawrenceshamboko

Burkitt lymphoma is an aggressive B-cell lymphoma associated with translocations involving the c-MYC gene. There are three main types: endemic, sporadic, and HIV-associated. Endemic Burkitt lymphoma most commonly presents as an abdominal mass in children in Africa and is strongly associated with Epstein-Barr virus. The tumor cells are intermediate in size with vacuolated cytoplasm, giving the characteristic "starry sky" appearance. Burkitt lymphoma is highly aggressive but responsive to chemotherapy.

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Mbarara University Of Science and Technology Lymphomas Pathology Department 2008
Learning Objectives Know the concept of Lymphoma Know the classification of lymphomas Know the most important morphological changes on lymphomas
What is Lymphoma? Name for a group of cancers of the lymphatic system, part of the body's immune defense system. It can occur when an error takes place in the way a lymphocyte is produced. The resulting abnormal cells accumulate either by duplicating faster or living longer than normal cells, and they displace normal lymphocytes.
Like normal lymphocytes, cancerous lymphocytes can grow in many parts of the body, including the lymph nodes, spleen, bone marrow or blood. Because there is lymph tissue throughout the body, the cancer cells may spread to other organs
Types of Lymphoma Hodgkin's Disease/Lymphoma (HD) Non-Hodgkin's Lymphoma (NHL)
Over the years, different classification systems have been used to differentiate lymphomas and several groups around the world are continuing to work on the best way to classify the various subtypes. Depending on the classification system being used, there are between 4 to 6 classes of HD and anywhere from 15 to over 30 classes of NHL
The simplest way to think of Lymphoma is to look at whether : It arises from a B- or T-cell lymphocyte (90% of lymphomas are of B-cell origin) it is a slow progressing lymphoma (indolent) or a fast progressing lymphoma (aggressive) All Hodgkins disease lymphomas are B-Cell lymphomas, so they can be further classified by classic or "lymphocyte predominant HD.
All Hodgkin's Disease lymphomas are B-Cell malignancies There are over 30 different NHL subtypes, classified by a combination of the two categories described
Lymphoma accounts for about 3 percent of all cases of cancer in the United States. Unfortunately, while the incidence rate of many other cancers is decreasing, the incidence rate for lymphoma is rising. Each year, 60,900 persons in the United States learn they have lymphoma. This figure includes approximately 7,000 new cases of Hodgkins disease (HD) and 53,900 new cases of Non-Hodgkins Lymphoma Incidence of Lymphoma
Facts about Lymphoma Non-Hodgkins Lymphoma is a much larger percentage of all lymphoma cases than Hodgkins Disease The pattern by age group varies for Hodgkins Disease vs. Non- Hodgkins Lymphoma
lymphomas(L).ppt
The incidence of Non-Hodgkins Lymphoma increases dramatically with age. Hodgkins Disease peaks among young adults (people in their mid-20s) falls to less than half that rate in middle age and increases infrequency in older adults
Lymphocyte Depletion Hodgkin's Disease (LDHD) Lymphocyte-Rich Classic Hodgkin's Disease (cLRHD) Mixed Cellularity Hodgkin's Disease (MCHD) Nodular Sclerosis Hodgkin's Disease (NSHD) Classic Hodgkins Disease
Classic Hodgkins Disease Lymphocyte Predominant Hodgkin's Disease (LPHD) Nodular Lymphocyte Predominant Hodgkins Disease (nLPHD) Diffuse Lymphocyte Predominant Hodgkins Disease (dLPHD)
Hodgkins Disease Reed Stemberg cell
Hodgkins Disease
Hodgkins Disease
Hodgkins Disease
Diffuse large B cell lymphoma (Immunoblastic lymphoma)
Small cell type, follicular lymphoma Nodular type
Small lymphocytic type, diffuse
NH lymphoma B cell Origin
Mbarara University of Science and Technology Pathology Department 2008
Denis P. Burkitt
Within his category fall: African (endemic) Burkitt Lymphoma Sporadic (nonendemic) Burkitt Lymphoma A subset of aggressive lymphomas occurring in individuals infected with HIV.
lymphomas(L).ppt
Burkitt Lymphomas occurring in each of these settings are histologically identical, but some clinical, genotypic, and virologic differences exist.
Morphology Involved tissue are effaced by a diffuse infiltrate of intermediate-sized lymphoid cells, 10-25 袖 in diameter, containing round or oval nuclei, and with coarse chromatin, several nucleoli and moderate amount of faintly basophilic or amphophilic cytoplasm.
The nuclear size approximates that of benign macrophages within the tumor. A high mitotic index is typical, as in apoptotic tumor cell death, accounting for the presence of numerous tissue macrophages with ingested nuclear debris. Morphology
These benign macrophages are diffusely ly distributed among the tumor cells and have abundant clear cell cytoplasm, creating a characteristic starry sky pattern. Morphology
In case with bone marrow involvement, the tumor cells in marrow aspirates have slightly clumped nuclear chromatin, two to five distinct nucleoli, and royal blue cytoplasm containing multiple, clear cytoplasmic vacuoles. Morphology
Immunophenotype These are tumors of mature B cells expressing surface IgM, monotypic or light chain, CD19. CD20, and CD10, and BCl6, a phenotype that closely resembles that of rapidly dividing B cells within the dark zones of normal germinal centers.
Cytogenetic and molecular Genetic Features All forms of Burkitt lymphomas are associated with translocations of the c-MYC gene on chromosome 8. The pattern in the translocation is usually the IgH occurs (t(8;14)) but may be also be the )(t:2;8)) or ((t(8;22)) light chain locus.
Essentially all endemic tumors are latently infected with EBV, which is also present in about 25% of HIV- associated tumors and 15% to 20% of sporadic cases. Cytogenetic and molecular Genetic Features:
Molecular analysis has shown that the configuration of the episomal EBV DNA, is identically in all tumors cells within the individual cases, indicating that infection precedes cellular transformation. Cytogenetic and molecular Genetic Features:
Clinical Features Both endemic and sporadic cases are found largely in children and young adults, accounting for approximately 30 % of childhood NHLs in the United State
Most tumors manifest at extranodal sites. Endemic Burkitt Lymphoma most often presents as an abdominal mass involving the ileocecum and peritoneum. Involvement of the bone marrow and peripheral blood is uncommon, specially in endemic cases. Burkitt Lymphoma is very aggressive but responds well to short-term high doses chemotherapy Clinical Features
Most tumors manifest at extranodal sites. Endemic Burkitt Lymphoma most often presents as an abdominal mass involving the ileocecum and peritoneum. Clinical Features
Involvement of the bone marrow and peripheral blood is uncommon, specially in endemic cases. Burkitt Lymphoma is very aggressive but responds well to short-term high doses chemotherapy Clinical Features
Patient and material collection In Uganda Population 95 cases from Ugandan tribes 2-64 years (median age: 16) Site of involvement: - lymph node (34 cases) - abdomen (26 cases) - gonads (25 cases) - jaw (10 cases) (Formalin fixed paraffin embedded blocks punched and collected in 2 TMA blocks (48 and 47 cases each) International Conference 2008, Kampala
Burkitt Lymphoma
lymphomas(L).ppt
Burkitt Lymphoma
Burkitt Lymphoma
Infected T cells EBV (immunoperoxidase stain for EBV)
Next topics Non Neoplastic Disease of Bone Tumor of Bone Inflammatory disease of joint Synovial Sarcoma Tumour of skeletal muscle Tumours of smooth muscle

More Related Content

lymphomas(L).ppt

  • 1. Mbarara University Of Science and Technology Lymphomas Pathology Department 2008
  • 2. Learning Objectives Know the concept of Lymphoma Know the classification of lymphomas Know the most important morphological changes on lymphomas
  • 3. What is Lymphoma? Name for a group of cancers of the lymphatic system, part of the body's immune defense system. It can occur when an error takes place in the way a lymphocyte is produced. The resulting abnormal cells accumulate either by duplicating faster or living longer than normal cells, and they displace normal lymphocytes.
  • 4. Like normal lymphocytes, cancerous lymphocytes can grow in many parts of the body, including the lymph nodes, spleen, bone marrow or blood. Because there is lymph tissue throughout the body, the cancer cells may spread to other organs
  • 5. Types of Lymphoma Hodgkin's Disease/Lymphoma (HD) Non-Hodgkin's Lymphoma (NHL)
  • 6. Over the years, different classification systems have been used to differentiate lymphomas and several groups around the world are continuing to work on the best way to classify the various subtypes. Depending on the classification system being used, there are between 4 to 6 classes of HD and anywhere from 15 to over 30 classes of NHL
  • 7. The simplest way to think of Lymphoma is to look at whether : It arises from a B- or T-cell lymphocyte (90% of lymphomas are of B-cell origin) it is a slow progressing lymphoma (indolent) or a fast progressing lymphoma (aggressive) All Hodgkins disease lymphomas are B-Cell lymphomas, so they can be further classified by classic or "lymphocyte predominant HD.
  • 8. All Hodgkin's Disease lymphomas are B-Cell malignancies There are over 30 different NHL subtypes, classified by a combination of the two categories described
  • 9. Lymphoma accounts for about 3 percent of all cases of cancer in the United States. Unfortunately, while the incidence rate of many other cancers is decreasing, the incidence rate for lymphoma is rising. Each year, 60,900 persons in the United States learn they have lymphoma. This figure includes approximately 7,000 new cases of Hodgkins disease (HD) and 53,900 new cases of Non-Hodgkins Lymphoma Incidence of Lymphoma
  • 10. Facts about Lymphoma Non-Hodgkins Lymphoma is a much larger percentage of all lymphoma cases than Hodgkins Disease The pattern by age group varies for Hodgkins Disease vs. Non- Hodgkins Lymphoma
  • 12. The incidence of Non-Hodgkins Lymphoma increases dramatically with age. Hodgkins Disease peaks among young adults (people in their mid-20s) falls to less than half that rate in middle age and increases infrequency in older adults
  • 13. Lymphocyte Depletion Hodgkin's Disease (LDHD) Lymphocyte-Rich Classic Hodgkin's Disease (cLRHD) Mixed Cellularity Hodgkin's Disease (MCHD) Nodular Sclerosis Hodgkin's Disease (NSHD) Classic Hodgkins Disease
  • 14. Classic Hodgkins Disease Lymphocyte Predominant Hodgkin's Disease (LPHD) Nodular Lymphocyte Predominant Hodgkins Disease (nLPHD) Diffuse Lymphocyte Predominant Hodgkins Disease (dLPHD)
  • 19. Diffuse large B cell lymphoma (Immunoblastic lymphoma)
  • 20. Small cell type, follicular lymphoma Nodular type
  • 22. NH lymphoma B cell Origin
  • 23. Mbarara University of Science and Technology Pathology Department 2008
  • 25. Within his category fall: African (endemic) Burkitt Lymphoma Sporadic (nonendemic) Burkitt Lymphoma A subset of aggressive lymphomas occurring in individuals infected with HIV.
  • 27. Burkitt Lymphomas occurring in each of these settings are histologically identical, but some clinical, genotypic, and virologic differences exist.
  • 28. Morphology Involved tissue are effaced by a diffuse infiltrate of intermediate-sized lymphoid cells, 10-25 袖 in diameter, containing round or oval nuclei, and with coarse chromatin, several nucleoli and moderate amount of faintly basophilic or amphophilic cytoplasm.
  • 29. The nuclear size approximates that of benign macrophages within the tumor. A high mitotic index is typical, as in apoptotic tumor cell death, accounting for the presence of numerous tissue macrophages with ingested nuclear debris. Morphology
  • 30. These benign macrophages are diffusely ly distributed among the tumor cells and have abundant clear cell cytoplasm, creating a characteristic starry sky pattern. Morphology
  • 31. In case with bone marrow involvement, the tumor cells in marrow aspirates have slightly clumped nuclear chromatin, two to five distinct nucleoli, and royal blue cytoplasm containing multiple, clear cytoplasmic vacuoles. Morphology
  • 32. Immunophenotype These are tumors of mature B cells expressing surface IgM, monotypic or light chain, CD19. CD20, and CD10, and BCl6, a phenotype that closely resembles that of rapidly dividing B cells within the dark zones of normal germinal centers.
  • 33. Cytogenetic and molecular Genetic Features All forms of Burkitt lymphomas are associated with translocations of the c-MYC gene on chromosome 8. The pattern in the translocation is usually the IgH occurs (t(8;14)) but may be also be the )(t:2;8)) or ((t(8;22)) light chain locus.
  • 34. Essentially all endemic tumors are latently infected with EBV, which is also present in about 25% of HIV- associated tumors and 15% to 20% of sporadic cases. Cytogenetic and molecular Genetic Features:
  • 35. Molecular analysis has shown that the configuration of the episomal EBV DNA, is identically in all tumors cells within the individual cases, indicating that infection precedes cellular transformation. Cytogenetic and molecular Genetic Features:
  • 36. Clinical Features Both endemic and sporadic cases are found largely in children and young adults, accounting for approximately 30 % of childhood NHLs in the United State
  • 37. Most tumors manifest at extranodal sites. Endemic Burkitt Lymphoma most often presents as an abdominal mass involving the ileocecum and peritoneum. Involvement of the bone marrow and peripheral blood is uncommon, specially in endemic cases. Burkitt Lymphoma is very aggressive but responds well to short-term high doses chemotherapy Clinical Features
  • 38. Most tumors manifest at extranodal sites. Endemic Burkitt Lymphoma most often presents as an abdominal mass involving the ileocecum and peritoneum. Clinical Features
  • 39. Involvement of the bone marrow and peripheral blood is uncommon, specially in endemic cases. Burkitt Lymphoma is very aggressive but responds well to short-term high doses chemotherapy Clinical Features
  • 40. Patient and material collection In Uganda Population 95 cases from Ugandan tribes 2-64 years (median age: 16) Site of involvement: - lymph node (34 cases) - abdomen (26 cases) - gonads (25 cases) - jaw (10 cases) (Formalin fixed paraffin embedded blocks punched and collected in 2 TMA blocks (48 and 47 cases each) International Conference 2008, Kampala
  • 45. Infected T cells EBV (immunoperoxidase stain for EBV)
  • 46. Next topics Non Neoplastic Disease of Bone Tumor of Bone Inflammatory disease of joint Synovial Sarcoma Tumour of skeletal muscle Tumours of smooth muscle