1. dodo Case 10 A 48 year old male initially presented with spinal cord compression and a paraspinal mass. He received local radiation therapy. Eight months later he developed generalized lymph node involvement and pancytopenia. Bone marrow and lymph node biopsies showed the presence of a lymphoma.

2. Lymph node

4. CD20

5. CD3

6. EMA PD-1

7. Case 10 LN Diagnosis: T-cell/histiocyte rich large B-cell lymphoma He received R-ESHAP, with partial response, followed by R-MINE. Splenomegaly was noted. He underwent splenectomy. Spleen weighed 2000 grams.

8. Spleen

11. CD20 CD3

12. OCT-2 PAX-5

13. PD-1/CD279

14. Case 10 Diagnosis: Spleen T-cell/ histiocyte rich large B-cell lymphoma, micronodular variant, involving the spleen CD20 negative secondary to rituximab therapy PD-1 rosettes raise a question as to whether this process is related to nodular lymphocyte predominant Hodgkin’s lymphoma

15. T-cell/Histiocyte-Rich Large B-cell Lymphoma May be challenging to distinguish from NLPHL with diffuse areas –Neoplastic cells resemble L&H cells –May have a vaguely nodular growth pattern –Immunophenotype similar to NLPHL (CD20+, EMA+) Clinically aggressive –Advanced clinical stage –Frequent BM involvement, hepatosplenomegaly –Poor response to therapy Stage IV NLPHL may be indistinguishable from TC/HRLBCL T-cell infiltration increases progressively in NLPHL over time

16. Variant Patterns in NLPHL Fan et al AJSP 2003

17. T-cell/ Histiocyte rich Large B-cell Lymphoma H&EEMA BCL-6

18. Histologic & Immunophenotypic Criteria NLPHL –Nodular or nodular and diffuse architecture –LP or “popcorn” cells –Expanded FDC meshworks with frequent small B-cells –Rosetting by PD-1+, CD57+ cells, CD4 > CD8 –CD20+, CD79a+, EMA+/-, CD30-, CD15- –Tumor cells associated with the lymphoid follicle T/HRLBCL –Diffuse architecture –Large neoplastic B-cells in a background of T-cells and/or histiocytes with absence of small B-cells –Paucity of FDC and CD57+ cells, CD8 > CD4 –CD20+, CD79a+, EMA+/-, CD30-, CD15-

19. Histological Progression in NLPHL Diffuse large B-cell lymphoma –5% of NLPHL –Clonally related by Ig gene PCR T-cell histiocyte rich large B-cell lymphoma –Controversial if these cases differ from de novo TCHRLBCL –Differences seen by CGH T-cell lymphomas (very rare)

20. CD20

21. NLPHL(LN) T/HRLBCL(BM)