1 / 18

Proposed WHO Classification of Lymphoid neoplasm

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).

meiying
Download Presentation

Proposed WHO Classification of Lymphoid neoplasm

An Image/Link below is provided (as is) to download presentation Download Policy: Content on the Website is provided to you AS IS for your information and personal use and may not be sold / licensed / shared on other websites without getting consent from its author. Content is provided to you AS IS for your information and personal use only. Download presentation by click this link. While downloading, if for some reason you are not able to download a presentation, the publisher may have deleted the file from their server. During download, if you can't get a presentation, the file might be deleted by the publisher.

E N D

Presentation Transcript

  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

More Related