Peripheral B cell Neoplasm Neoplasm of Mature B cells<br />
FOLLICULAR LYMPHOMA <br />
FEATURES<br />Follicular lymphoma is the most common form of indolent NHL in the United States<br />Middle age men and wom...
hallmark<br />Translocation( 14; 18) <br />This translocation is seen in most but not all follicular lymphomas<br />Leads ...
Features<br />10% show Peripheral blood involvement sufficient to produce lymphocytosis<br />	(usually <20,000/mm3 )<br />...
Reactive LymphoidFollicular Lymphoma  	Hyperplasia<br />Majority Small cleaved cells<br />Form Follicles<br />
BCL2 Immunostain<br />7<br />
Immunophenotype and Genetics<br />Express CD19, CD20, CD10 <br />Like Normal follicular center B cells,<br />CD5 is NOT Ex...
Clinical Features.<br />Painless lymphadenopathy, which is frequently generalized. <br />Uncommon Involvement of extranoda...
Survival<br />Overall median survival is 7 to 9 years<br />Is not improved by aggressive therapy<br />The usual clinical a...
Transformation<br />Retain t(14;18)<br />Somatic Hypermutation promote transformation<br />Occurs in 30 to 50% of follicul...
DIFFUSE LARGE CELL LYMPHOMA<br />
Most common form of NHL<br />60-70% <br />Aggressive lymphoid neoplasm<br />M>F , Median age 60y/o<br />DIFFUSE LARGE B-CE...
Features<br />Rapidly enlarging mass<br />Often Symptomatic<br />Arise in any site<br />Waldeyerring, Oropharyngeal LN, To...
Immunophenotype<br />Mature B cell <br />Express CD19 & CD20<br />Variably Express Germinal Center Markers<br />Have surfa...
Molecular Pathogenesis<br />30% Dysregulation of BCL6<br />Repress germinal center B-cell Differentiation  Growth Arrest ...
Liver -DLCL<br />
Morphology<br />Diffuse pattern of growth<br />Large Neoplastic cells<br />4-5x small lymphocytes<br />DIFFUSE LARGE CELL ...
Diffuse Large Cell<br />
Therapy<br />60-80% Complete remission with combination Chemotherapy<br />50% remain free from disease for years<br />Immu...
Subtype <br />Immunedeficiency-associated large B cell Lymphoma<br />T cell immunodeficiency ( HIV )<br />(+) EBV Neoplast...
BURKITTS LYMPHOMA<br />
BURKITTS LYMPHOMA<br />3 TYPES<br />1. African ( Endemic )<br />2. Sporadic ( Non-endemic )<br />3. Aggressive lymphoma oc...
Genotype & virologic difference
CD10 Usually seen</li></li></ul><li>Features<br /><ul><li>Cell of origin
Germinal center Bcell
African  LATENTLY INFECTED w/ EBV
All forms associated
Translocations c-myc gene on Chromosome 8 with IgH [t(8,14)]
Commonly</li></li></ul><li>Clinical features<br />Adolescent or Young Adult w/ jaw or extranodal abdominal mass<br />Very ...
Clinical features<br />   Endemic	<br />Often as Abdominal Mass<br />Ileocecal<br />Peritoneum<br />Often Mandibular mass<...
Morphology<br />Starry sky pattern<br />High mitotic activity<br />
BurkittsLymphomaStarry sky pattern<br />
High Mitotic IndexMonotonous Cells<br />
Marginal Zone Lymphomas<br />
Features<br />LOW grade lesions<br />Encompass a heterogenous group of 	B cell tumors<br />Arise in LN, Spleen, Extranodal...
Unusual Pathogenesis<br />1. Often arise – Chronic Inflammatory D/O<br />Autoimmune<br />Sjogrens – Salivary gland<br />Ha...
Unusual Pathogenesis<br />2. Remain localized for prolonged periods – Spread late<br />3. May regress if inciting agent is...
GIT- Maltoma<br />
MULTIPLE MYELOMA<br />
Multiple bone involvement<br />Can also spread to LN & Extranodal<br />1% in Western countries<br />Higher incidence <br /...
Pathogenesis<br />IL-6 <br />Proliferation of tumor cells are DEPENDENT on Cytokione<br />Active Disease and Poor Prognosi...
X-ray<br />Multiple lyticlesions <br />Punch out lesions<br />Axial Skeleton<br />Starts at Medullary<br />Gelatinous , so...
Mutiple Myeloma<br />
Laboratory<br />High M proteins  Rouleaux<br />55% IgG Monoclonal Ab<br />Proliferation of Neoplastic plasma cells<br />3...
BM aspirate- Myeloma<br />
Electrophoresis<br />IgG k M protein<br />
Clinical Features<br />Bone pain – axial skeleton (Vertebrae)<br />Hypercalcemia ( 25%)<br />Renal Failure (30-50%)<br />M...
Clinical Features<br />Hematologic findings<br />Normocytic anemia with rouleaux<br />Prolonged bleeding due to defect in ...
Prognosis<br />Variable but Generally Poor<br />Median survival is 6 months without treatment<br />
SOLITARY MYELOMA<br />
Solitary Myeloma<br />Lesions either in the Bone or Soft Tissue<br />Axial Skeleton<br />Lungs, Oropharynx, Nasal Sinuses<...
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Part 2 Nhl

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Part 2 Nhl

  1. 1. Peripheral B cell Neoplasm Neoplasm of Mature B cells<br />
  2. 2. FOLLICULAR LYMPHOMA <br />
  3. 3. FEATURES<br />Follicular lymphoma is the most common form of indolent NHL in the United States<br />Middle age men and women equally.<br />Arise from germinal center B cells.<br />Strongly associated with translocation involving BCL2<br />
  4. 4. hallmark<br />Translocation( 14; 18) <br />This translocation is seen in most but not all follicular lymphomas<br />Leads to overexpression of BCL2 protein.<br /> BCL2, = is an antagonist of apoptotic cell death and appears to promote the survival of follicular lymphoma cells. <br />
  5. 5. Features<br />10% show Peripheral blood involvement sufficient to produce lymphocytosis<br /> (usually <20,000/mm3 )<br />85% have Bone marrow involvement <br />Paratrabecularlymphoid aggregates.<br />Splenic white pulp and hepatic portal triads are also frequently involved.<br />
  6. 6. Reactive LymphoidFollicular Lymphoma Hyperplasia<br />Majority Small cleaved cells<br />Form Follicles<br />
  7. 7. BCL2 Immunostain<br />7<br />
  8. 8. Immunophenotype and Genetics<br />Express CD19, CD20, CD10 <br />Like Normal follicular center B cells,<br />CD5 is NOT Expressed <br />In contrast to CLL and SLL and mantle cell lymphoma, CD5 is expressed. <br />OverExpression of BCL2 protein - > 90%<br />Versus Normal Follicular center B cells, which are BCL2 negative <br />
  9. 9. Clinical Features.<br />Painless lymphadenopathy, which is frequently generalized. <br />Uncommon Involvement of extranodalsites<br />GIT, CNS, Testis<br />Often follows an indolent waxing and waning course. <br />
  10. 10. Survival<br />Overall median survival is 7 to 9 years<br />Is not improved by aggressive therapy<br />The usual clinical approach is to palliate patients with low-dose chemotherapy or radiation when they become symptomatic. <br />
  11. 11. Transformation<br />Retain t(14;18)<br />Somatic Hypermutation promote transformation<br />Occurs in 30 to 50% of follicular lymphomas, <br />Most commonly to diffuse large B-cell lymphoma. <br />Median survival is less than 1 year after transformation.<br />
  12. 12. DIFFUSE LARGE CELL LYMPHOMA<br />
  13. 13. Most common form of NHL<br />60-70% <br />Aggressive lymphoid neoplasm<br />M>F , Median age 60y/o<br />DIFFUSE LARGE B-CELL LYMPHOMA<br />
  14. 14. Features<br />Rapidly enlarging mass<br />Often Symptomatic<br />Arise in any site<br />Waldeyerring, Oropharyngeal LN, Tonsils<br />Liver, spleen<br />Localized Disease with extranodal involvement <br />Rarely present as leukemia<br />
  15. 15. Immunophenotype<br />Mature B cell <br />Express CD19 & CD20<br />Variably Express Germinal Center Markers<br />Have surface Ig<br />Negative Tdt<br />
  16. 16. Molecular Pathogenesis<br />30% Dysregulation of BCL6<br />Repress germinal center B-cell Differentiation  Growth Arrest  Holds cell in Undifferentiated Proliferative state<br />Silence the expression of p53 <br />Prevent the activation of DNA repair mechanism<br />
  17. 17. Liver -DLCL<br />
  18. 18. Morphology<br />Diffuse pattern of growth<br />Large Neoplastic cells<br />4-5x small lymphocytes<br />DIFFUSE LARGE CELL LYMPHOMA<br />
  19. 19. Diffuse Large Cell<br />
  20. 20. Therapy<br />60-80% Complete remission with combination Chemotherapy<br />50% remain free from disease for years<br />Immunotherapy with Anti-CD20 improves outcome especially elderly <br />
  21. 21. Subtype <br />Immunedeficiency-associated large B cell Lymphoma<br />T cell immunodeficiency ( HIV )<br />(+) EBV Neoplastic B cell <br />Restoration of immunity <br />Regression of proliferation <br />
  22. 22. BURKITTS LYMPHOMA<br />
  23. 23. BURKITTS LYMPHOMA<br />3 TYPES<br />1. African ( Endemic )<br />2. Sporadic ( Non-endemic )<br />3. Aggressive lymphoma occuring in HIV patients<br /><ul><li>Histologically identical
  24. 24. Genotype & virologic difference
  25. 25. CD10 Usually seen</li></li></ul><li>Features<br /><ul><li>Cell of origin
  26. 26. Germinal center Bcell
  27. 27. African  LATENTLY INFECTED w/ EBV
  28. 28. All forms associated
  29. 29. Translocations c-myc gene on Chromosome 8 with IgH [t(8,14)]
  30. 30. Commonly</li></li></ul><li>Clinical features<br />Adolescent or Young Adult w/ jaw or extranodal abdominal mass<br />Very aggressive<br />Respond well to chemotx<br />Outcome guarded in Older adults<br />UNCOMMON BM or peripheral blood<br />
  31. 31. Clinical features<br /> Endemic <br />Often as Abdominal Mass<br />Ileocecal<br />Peritoneum<br />Often Mandibular mass<br />Unusual predilection to abdominal viscera<br />Kidneys<br />Ovary<br />Adrenals<br />Sporadic<br />
  32. 32.
  33. 33.
  34. 34. Morphology<br />Starry sky pattern<br />High mitotic activity<br />
  35. 35. BurkittsLymphomaStarry sky pattern<br />
  36. 36. High Mitotic IndexMonotonous Cells<br />
  37. 37. Marginal Zone Lymphomas<br />
  38. 38. Features<br />LOW grade lesions<br />Encompass a heterogenous group of B cell tumors<br />Arise in LN, Spleen, Extranodal Tissues<br />Tumor cell resemble normal Marginal Zone B cells<br />Initially recognized at mucosal sites <br />MALTOMA<br />
  39. 39. Unusual Pathogenesis<br />1. Often arise – Chronic Inflammatory D/O<br />Autoimmune<br />Sjogrens – Salivary gland<br />Hashimotos - Thyroid<br />Infectious <br />Helicobacter pylori- Stomach<br />
  40. 40. Unusual Pathogenesis<br />2. Remain localized for prolonged periods – Spread late<br />3. May regress if inciting agent is eradictaed – H. pylori<br />
  41. 41.
  42. 42.
  43. 43. GIT- Maltoma<br />
  44. 44.
  45. 45. MULTIPLE MYELOMA<br />
  46. 46. Multiple bone involvement<br />Can also spread to LN & Extranodal<br />1% in Western countries<br />Higher incidence <br />Men>Women<br />Older Patients<br />Radiation exposure<br />African decent<br />MULTIPLE MYELOMA<br />
  47. 47. Pathogenesis<br />IL-6 <br />Proliferation of tumor cells are DEPENDENT on Cytokione<br />Active Disease and Poor Prognosis<br />MIP 1 alpha & RANK Ligand<br />Mediate Bone Destruction<br />Karyotyping<br />Deletions of 13q<br />IgH<br />MULTIPLE MYELOMA<br />
  48. 48. X-ray<br />Multiple lyticlesions <br />Punch out lesions<br />Axial Skeleton<br />Starts at Medullary<br />Gelatinous , soft tumor<br />MULTIPLE MYELOMA<br />
  49. 49. Mutiple Myeloma<br />
  50. 50. Laboratory<br />High M proteins  Rouleaux<br />55% IgG Monoclonal Ab<br />Proliferation of Neoplastic plasma cells<br />30% of bone marrow cellularity<br /> (Plasma cell Leukemia )<br />Bence Jones proteins in urine<br />Myeloma kidney<br />Seen in 60-80%<br />ClinicopathologicDx<br />Correlation of X-ray & Laboratory Findings<br />MULTIPLE MYELOMA<br />
  51. 51. BM aspirate- Myeloma<br />
  52. 52. Electrophoresis<br />IgG k M protein<br />
  53. 53. Clinical Features<br />Bone pain – axial skeleton (Vertebrae)<br />Hypercalcemia ( 25%)<br />Renal Failure (30-50%)<br />Myeloma kidney<br />Proteinacious tubular cast<br />Nephrocalcinosis ( metastatic calcification)<br />
  54. 54. Clinical Features<br />Hematologic findings<br />Normocytic anemia with rouleaux<br />Prolonged bleeding due to defect in platelet aggregation<br />Radiculopathy due to bone compression and vertebral fracture<br />Recurrent infection – Most common cause of death<br />
  55. 55. Prognosis<br />Variable but Generally Poor<br />Median survival is 6 months without treatment<br />
  56. 56. SOLITARY MYELOMA<br />
  57. 57. Solitary Myeloma<br />Lesions either in the Bone or Soft Tissue<br />Axial Skeleton<br />Lungs, Oropharynx, Nasal Sinuses<br />Minority show (+) M protein<br />Progression to Multiple Myeloma<br />Common in solitary Osseous myeloma <br /> ( 10-20 yrs)<br />Less common in Extraosseous<br />
  58. 58. PERIPHERAL T-CELL and NK-CELL NEOPLASMS<br />NEOPLASM OF MATURE T CELLS AND NK CELLS<br />
  59. 59. EXTRANODAL NK/T-CELL LYMPHOMA<br />
  60. 60. EXTRANODAL NK/T-CELL LYMPHOMA<br />PREVIOUSLY MIDLINE GRANULOMA<br />3% OF NHL IN ASIA<br />DESTRUCTIVE MIDLINE MASS  INVADE SMALL VESSELS  EXTENSIVE ISCHEMIC NECROSIS<br />NEOPLASTIC ELEMENTS<br />MIXTURE OF SMALL & LARGE LYMPHOID CELLS<br />
  61. 61.
  62. 62. Mycosis fungoides<br />
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