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Proposed WHO Classification of Lymphoid neoplasm PowerPoint Presentation
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Proposed WHO Classification of Lymphoid neoplasm

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Proposed WHO Classification of Lymphoid neoplasm - PowerPoint PPT Presentation

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Proposed WHO Classification of Lymphoid neoplasm

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  1. Proposed WHO Classification of Lymphoid neoplasm B cell neoplasm Precursor B cell neoplasm Precursor B-lymphoblastic leukemia/lymphoma (precursor B-ALL) T cell neoplasm Precursor T-cell and NK-cell neoplasm Precursor T-lymphoblastic lymphoma/leukemia (precursor T-ALL)

  2. Mature (peripheral) B cell neoplasm B-cell chronic lymphocytic leukemia/SLL Lymphoplasmacytic lymphoma Splenic marginal zone B-cell lymphoma Hairy cell leukemia Plasma cell myeloma/plasmacytoma Extra-nodal marginal zone B cell lymphoma of MALT type Nodal marginal zone B cell lymphoma Follicular lymphoma Mantle cell lymphoma

  3. Mature (peripheral) B cell neoplasm (cont’d) Diffuse large B cell lymphoma Mediastinal B cell lymphoma Primary effusion lymphoma Burkitt lymphoma/Burkitt cell leukemia

  4. Mature (peripheral) T cell neoplasm T-cell prolymphocytic leukemia T-cell granular lymphocytic leukemia Aggressive NK-cell leukemia Adult T-cell leukemia/lymphoma (HTLV1) Extranodal NK/T cell lymphoma, nasal type Enteropathy type T cell lymphoma Hepatosplenic gamma delta T cell lymphoma Subcutaneous panniculitis-like T cell lymphoma Mycosis fungoides/Sezary syndrome

  5. Mature (peripheral) T cell neoplasm (cont’d) Anaplastic large cell lymphoma, T/null cell, primary cutaneous Peripheral T cell lymphoma, NOS Angioimmunoblastic T cell lymphoma Anaplastic large cell lymphoma, T/null cell, primary systemic

  6. Hodgkin's lymphoma (Hodgkin's disease) Nodular lymphocyte predominant Hodgkin's lymphoma Classical Hodgkin's lymphoma Nodular sclerosis Hodgkin's lymphoma (grades 1 and 2) Lymphocyte-rich classical Hodgkin's lymphoma Mixed cellularity Hodgkin's lymphoma Lymphocyte depletion Hodgkin's lymphoma

  7. Pediatric Follicular Lymphoma • Rare lymphoma subtype in children (1-2%) • 60% present in head and neck • Tonsils, nasopharynx • Other sites: lymph nodes, GI tract, testis Many differences with FL in adults • Usually Grade II-III • Bcl-2 usually negative (both protein & bcl-2R) • Male: Female ratio 3:1 • 85% present with Stage I or II disease • 75% CR with low relapse rate

  8. LBCL BL Children LBCL BL Adults

  9. Burkitt-like lymphoma should exist? Burkitt-like lymphoma should be included in: Diffuse large B cell lymphoma or Morphologic variant of Burkitt lymphoma

  10. Burkitt and Burkitt-like Lymphomas May show follicular colonization Cytologically tumor cells resemble the small blasts of the dark zone of the GC High rate of somatic mutations of Ig VH BCL-6+, CD 10+ Evidence favors a germinal center derivation

  11. Burkitt-like Lymphomas • Similar to BL, but with greater variation in nuclear morphology • MIB-1 100%, CD10 +, bcl-2 - • Cytogenetics or molecular genetics desirable for diagnosis - should have a c-myc translocation • This morphologic variant more common in immunodeficiency states

  12. Intermediate morphology C-myc translocations High proliferative fraction In the WHO classification The term “Burkitt-like lymphoma” is retained Burkitt-like lymphoma will be considered a variant of Burkitt lymphoma Major criteria:

  13. Atypical Burkitt/ Burkitt- like With plasmacytoid differentiation (HIV -associated) BURKITT LYMPHOMA Morphologic Variants Classical Burkitt lymphoma Subtypes, clinical genetic Endemic Sporadic Immunodeficiency-associated

  14. Anaplastic Large Cell Lymphoma • Paradigm for process used to define disease entities (REAL) • First recognized based on • Morphology - sinusoidal growth • Antigenic phenotype - CD30+ • Studies of molecular pathogenesis led to new diagnostic tools • RT-PCR and ALK-1 monoclonal antibody • New diagnostic tools define the borderlands of the disease

  15. Anaplastic Large Cell LymphomaClinical Features • Presents most commonly in lymph nodes • Cutaneous involvement variable • Most common in children, young adults • Frequent presence of systemic symptoms • “B” symptoms or high IPI less often predict poor outcome (in contrast to most other NHL) • Aggressive natural history but good response to chemotherapy

  16. DISTRIBUTION OF ANAPLASTIC LARGE CELL LYMPHOMA BY AGE (267 cases) Sex Ratio= 1.34 Number of cases Age

  17. 100 ALK+ (n=132) Survival of 154 patients with ALCL Survival ALK - (n=22) 75 p<0.001 50 25 0 60 120 180 240 Months

  18. Clinical Significance of ALK + in Anaplastic Large Cell Lymphoma • ALK+ cases are seen in younger age groups • Children and young adults; M>>F • ALK+ cases have better prognostic than ALK-, irrespective of other clinical features; i.e. age, stage or IPI Conclusion: • ALK+ ALCL is probably a distinct disease entity, separate from ALK- ALCL