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AP/CP

AP/CP. Presenter Zulfiya Ibragimova MD PGY-1 Special thanks to Dr. Venkataraman and Dr. Li March 29, 2012. Clinical history. A 73-year-old female with fatigue, abdominal discomfort, night sweats was admitted for evaluation. CT : lesion at the root of the mesentery measuring 3.4 x 2 cm

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AP/CP

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  1. AP/CP Presenter Zulfiya Ibragimova MD PGY-1 Special thanks to Dr. Venkataraman and Dr. Li March 29, 2012

  2. Clinical history • A 73-year-old female with fatigue, abdominal discomfort, night sweats was admitted for evaluation. • CT : lesion at the root of the mesentery measuring 3.4 x 2 cm • additional adenopathy in the region including the portocaval region. • On 01/17/2012, she underwent a laparoscopic resection of the mesenteric mass.

  3. H&E x 2

  4. H&E x 10

  5. H&E x 40

  6. Differential diagnosis? • T-cell process: -Angioimmunoblastic T-cell lymphoma (effaced architecture, hypervascular stroma, few large cells in a small lymphoid background • B-cell process: -Hodgkin lymphoma (cHL) -classical - nodular lymphocyte-predominant Hodgkin lymphoma (NLPHL) -T-cell/histiocyte rich large B-cell lymphoma (THRLBCL) -EBV+ lymphoproliferative disorder of elderly (EBV-LPD)

  7. What can we do to work up this case? • IHC • Flow cytometry • PCR (gene rearrangement) • Cytogenetics • Special studies

  8. Classical Hodgkin lymphoma Monoclonal B-cell neoplasm • neoplastic Hodgkin cells, multinucleated Reed-Sternberg cells • non-neoplastic background of small lymphocytes, eosinophils, histiocytes, plasma cells, fibroblasts • Most US cHLs are of the NS subtype. • Less likely to be EBV-driven (10-20% of cases) compared to eastern cHL which is MC subtype and nearly 70% EBV-associated. • Bimodal distribution: 15-35 years and later in life • Location: nodal.

  9. Classical Hodgkin lymphoma morphology Large cells: CD30+ CD15+/- Mum+ wPax5+ CD20-/+ EBV- IgD- CD45- Background: T-cells (CD4>CD8) eosinophils histiocytes fibroblasts EBER,LMP

  10. not cHL Our case H&E and IHC CD20 Pax5 Mum1 CD30 CD15 CD23 EBV-LMP EBER

  11. Angioimmunoblastic lymphoma AITL • systemic disease • polymorphous infiltrate • prominent proliferation of high endothelial venules and follicular dendritic cells • 1-2% of all non-Hodgkin lymphomas, 15-20% of all T-cell lymphomas • Male : female = 1:1, middle-aged and elderly • Location: nodal (always) and frequent extranodal involvement • Prognosis: aggressive, median survival < 3 years • Treatment: Chemotherapy. The best approach is still unknown.

  12. Angioimmunoblastic lymphoma

  13. Angioimmunoblastic lymphoma morphology • Architecture is effaced • Small cells (the bad guys): clear cytoplasm with distinct cell membranes and cytological atypia • Large cells: Immunoblasts-like, RS-like, scattered • High endothelial venules • Reactive background

  14. Angioimmunoblastic lymphoma morphology: lymphoma cells and large cells CD4 CD10 PD 1 CD30 CD15 EBER

  15. Angioimmunoblastic lymphoma morphology: background CD8 CD21 CD20 CD20

  16. Our case H&E and IHC not AITL Our case AITL CD20 EBER CD23 CD30 CD15 TCR PCR showed polyclonal TCR gene rearrangement

  17. EBV-LPD of elderly (EBV-positive diffuse large B-cell lymphoma (DLBCL) of elderly ) EBV+ clonal lymphoid proliferation in patients > 50 y/o old without any know immunodeficiency or prior lymphoma • In Asia it accounts for 8-10% of all DLBCL • Little data in Western countries • Male : female = 1.5 : 1.0 • Location: 70% extranodal with/without simultaneous lymph nodes involvement 30% lymph nodes alone • Prognosis: poor, median survival is 2 years • Treatment: R-CHOP, Radiation or combination of both. Bone marrow transplantation is possible.

  18. EBV-LPD of elderly: Morphology • Architecture is effaced • Large cells (the bad guys): Hodgkin-like, RS-like, blasts-like scattered or in clusters • Reactive background

  19. EBV-LPD of elderly: Morphology Architecture is effaced Large cells monoclonal: Hodgkin-like and RS-like, blasts-like • sCD20+ (key point to distinguish from cHL) • sCD30+ • PAX5+ • Oct2+ • Mum1+ • EBER+ (small and large cells- in contrast to cHL-only large cells) • CD15-/+ • CD3 and CD4- • Kappa/Lambda negative or restricted • No genetic translocations specific for this process found Reactive background: small B-cells, T-cells(CD4, CD8), plasma cells, histiocytes, epithelioid cells. T cells may be clonal* or restricted (detected by PCR)

  20. EBV-LPD of elderly: Differential diagnosis • Differential with cHL: -Both may be wCD20, CD30+, CD 15+ -Not based on CD15 expression only -Should consider extranodal site – very rare for cHL -EBV+ only in large cells in cHL -EBV-LPD EBV+ seen in greater number and range of cells** • Differential with AITL: -Both have numerous EBV+ cells -Both may have clonal T-cells -Should look for appropriate phenotype of these T cells (CD10/CD4/CXCL13/PD1+ Tfh) -AITL has expanded extrafollicular dendritic cell meshworks ** Dojcinov S, Venkataraman G, Pittaluga S, Jaffe ES, et al. 2011 Age-related EBV-associated lymphoproliferative disorders in the Western population: a spectrum of reactive lymphoid hyperplasia and lymphoma. Blood 117:4726-35

  21. EBV-LPD of elderly: Morphology –Large cells CD30 CD20 Mum1 wPax5 EBV-LMP EBER

  22. EBV-LPD of elderly: Morphology –Large cells and background CD15 Kappa Lambda CD3 CD4 CD8 CD20

  23. Our case IHC (looking at large cells) not EBV-LPD CD20 CD30 CD15 Mum1 EBER EBV-LMP

  24. NLPHL and THRLBCL

  25. NLPHL and THRLBCL

  26. NLPHL and THRLBCL CD20 CD20 CD20 CD20 CD21 CD4/CD57 CD8/TIA1 CD4 CD8 CD21 Histopathology(2002) 41, 216–229

  27. Our case IHC (large cells) CD20 CD20 Pax5 Oct2 EBV-LMP CD79a CD15 CD30

  28. Our case IHC (background) Not NLPHL CD4 CD8 CD20 CD23

  29. Final diagnosis • Aggressive B-cell lymphoma most consistent with a T-cell/histiocyte rich large B-cell lymphoma Comment: While the marked preponderance of CD4 T-cells over CD8 is unusual for T/HRLBCL, we consider this as the best subclassification for this lymphoma

  30. References • Fraga M, Garcı´a-Rivero A, Sa´nchez-Verde L, Forteza J & Piris M A (2002) T-cell ⁄ histiocyte-rich large B-cell lymphoma is a disseminated aggressive neoplasm:differential diagnosis from Hodgkin’s lymphoma Histopathology 41, 216–229 • Peter Van Loo,1,2,3 Thomas Tousseyn,4 Vera Vanhentenrijk,4 Daan Dierickx,5 Agnieszka Malecka,6 Isabelle Vanden Bempt,4 Gregor Verhoef,5 Jan Delabie,6 Peter Marynen,1,2 Patrick Matthys,7 and Chris De Wolf-Peeters4 (2010) T-cell/histiocyte-rich large B-cell lymphoma shows transcriptional features suggestive of a tolerogenic host immune response Haematologica 95(3):440-448 • Tousseyn T, De Wolf-Peeters C (2011) T cell/histiocyte-rich large B-cell lymphoma: an update on its biology and classification. Virchows Arch 459(6):557-63 • Shimoyama Y, Oyama T, Asano N, Oshiro A, Suzuki R, Kagami Y, MorishimaY, Nakamura S (2006)Senile Epstein-Barr virus-associated B-cell lymphoproliferative disorders: a mini review. J Clin Exp Hematop. 46(1):1-4 • Dojcinov S, Venkataraman G, Pittaluga S,Jaffe ES, et al. 2011 Age-related EBV-associated lymphoproliferative disorders in the Western population: a spectrum of reactive lymphoid hyperplasia and lymphoma. Blood 117:4726-35

  31. THANK YOU

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