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EAHP 16- LYWS-217 BASEL

This case report presents an aggressive form of follicular lymphoma (FL) with a translocation (8;14), leading to a rapidly growing tumor. The patient experienced an abrupt onset of symptoms and had a markedly aggressive course. The pathology findings, genetic studies, and clinical history are detailed.

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EAHP 16- LYWS-217 BASEL

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  1. EAHP 16- LYWS-217 BASEL Follicularlymphoma grade 3B with t(8;14)

  2. Ludmila Boudova* 1, Pavla Veselá1, Michal Karas2, Petr Grossmann1, Jana Kuntscherova1, Pavel Jindra2, Tomas Vanecek1, Petr Mukensnabl1, David Slouka, Michal Michal1, Ivan Subrt3, Marketa Stenova3 1Pathology, 2Hematooncology, 3Genetics, Charles University, MedicalFacultyHospital, Pilsen, Czech Republic

  3. Clinical history A woman, 54 ys. Past history: ulcerouscolitis (dg. 2011), mesalazine, no immunosuppression Presentedwithanabruptonsetofsymptoms and a markedlyaggressivecourse April 2014 - enlargedcervical LN and rapidlygrowing bulky abdominaltumours (9 cm in thediam), massivegeneralizedlymphadenopathy pleuraleffusion,  massive BM infiltration, high LDH, IPI3, stage IV.B

  4. Therapy and outcome Aggressive: R-HyperCVAD, R-HIDAC + MTX, BEAM withautologous PBSCT, CHOP,  2 completeremissions a relapse in the oral cavity (gingiva) in her second CR - allogenic PBSCT Shediedofpneumonia in December 2015.

  5. Firstbiopsy – LN -distinctfolliculararchitecture, no DLBCL component

  6. Follicular architecture and starry-sky pattern

  7. Neoplastic „starry sky“follicle without any mantle, interfollicular small reactive lymphocytes

  8. Centroblasts, centrocytes,highmitotic and apoptoticactivity

  9. Immunohistochemistry LN - neoplastic cells • Positive: CD20, CD79a, PAX5, CD10, bcl6, bcl2, p53, c-myc; Ki67: approaching 100% • Negative:cyclin D1, TdT, MUM1, CD34, CD30; IgL: slightly more kappa; polyclonalheavychains • folliculardendriticmeshworksretainedonly in fewfollicles(CD23); a smalladmixtureof T-cells, mainlyaroundfollicles (CD3, CD5) –helping make thefolliculararchitecturevery distinct

  10. CD20 bcl2

  11. CD10 BCL6

  12. CD23

  13. MUM1 CD5

  14. Ki67

  15. C-myc Lymph node C-myc p53

  16. Bone marrow (28 May 2014)denseinterstitial to diffuseinfiltration p53 bcl2

  17. C-myc CD20 Bone marrow diffuseinfiltration by large B-cells positive: CD20, CD79a, bcl2, bcl6, p53, c-myc; Ki67 over 95%; negative: TdT

  18. Gingiva – relapse (May 2015) c-myc p53

  19. Genetic studies • Cytogenetics: • lymph node (FFPE) - FISH - positive: t(8;14); negative: t(11;14); bcl6; • repeatedly not assessable (breakapart, fusionprobes): t(14;18) • similar: gingiva (FFPE) – relapse • Bone marrow (fresh; examined in VI-VII/2014): • G-banding and FISH:  tumourcells: t(14;18);t (8;14); del TP53, chrom. 7 trisomy • Molecularstudies: PCR • BM - fresh - bcl2/JHa : positive (mcr); IgH: clonal • LN: bcl2/Jha(mbr, mcr): negative; IgH: clonal • IgH and IgKproducts – comparison • samesize in allthreesamples - IgK – 285 bp • IgH – (FR1 – 334 bp, FR2 – 267 bp), FR3 – 133 b • EBER ISH, EBV (PCR) – all negative (LN, BM, gingiva)

  20. Proposed diagnosis: Lymphnode: follicularlymphoma grade 3B, with a folliculararchitecture- witht(8;14); othertranslocations not detectedpossiblybecauseof a poortechnicalqualityofthe FFPE sample Bone marrow: diffuselarge B-cell lymphomawitht (8;14), t(14;18) del TP 53. chrom.7 trisomy- transformationofFL Gingiva – relapse of DLBCL

  21. Interesting feature(s) of submitted case: • Clinical: an abrupt onset of symptoms – • a very aggressive presentation, rapidly growing large tumours (“growing like a Burkitt lymphoma…“) • Pathological:follicular lymphoma grade 3B without DLBCL – as found in the initial LN is relatively rare • t(8;14) in FL g. 3B is rare - found in 13-20%; found in a similar percentage of FL of all grades • (Horn 2011, Bosga – Bouwer; Au, Mohamed; Offit)

  22. C-mycbreaks in FL rare • 40% oflymphomaswithdualmyc/bcl2 have a historyof FL • 50% oflymphomaswith c-mycrearrangement are DHL – DLBCL, Burkitt, to a lesserextent FL • c-myc - involved in transformation maybeanadverseprognosticindicator.  Differentialdiagnosis: • FL, blastoid and FL 3B shoulddiffer in the cytology oftumourcells, but sharesomeimmunomorphological and geneticfeatures (e. g. starryskypattern, very highmitotic and proliferativerate, somegeneticchanges) • Burkittlymphomawith a folliculargrowthpattern

  23. Transformation – possible scenarios: • FL isusuallyknown to havebeenpresentforyearsbeforeittransforms to DLBCL. Thetransformationof FL  occurs in 3% ofcases per yearoverthefirst 15 ysofobservation, most commonly to DLBCL 2. Theremaybepatientswhohave LN with FL forsometime and go undiagnoseduntilthetransformation - whichmaydrawattention by clinicalworsening

  24. Remark: • Ourpatientwent to see her doctorsfrequently ( gastroenterologist; gymaecologist) yearsbeforethepresentationoflymphoma Thelymphomaprobablystarted to grow very rapidlywhichisunusual but wecannotgenerallyexcludethatsomelymphnodeswith a lymphoma in anindolentphasemayhaveescapedmedicalattention

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