Case presentation 18 frontiers 2011
Download
1 / 27

Case Presentation #18 Frontiers 2011 - PowerPoint PPT Presentation


  • 62 Views
  • Uploaded on

Case Presentation #18 Frontiers 2011. Lauren B. Smith, M.D. Hematopathology. Clinical History: 54 year-old man. 10/2001. Lymphocytosis Splenomegaly Adenopathy. Femoral lymph node biopsy October 2001 (H&E, 20X magnification ).

loader
I am the owner, or an agent authorized to act on behalf of the owner, of the copyrighted work described.
capcha
Download Presentation

PowerPoint Slideshow about ' Case Presentation #18 Frontiers 2011' - ohio


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.While downloading, if for some reason you are not able to download a presentation, the publisher may have deleted the file from their server.


- - - - - - - - - - - - - - - - - - - - - - - - - - E N D - - - - - - - - - - - - - - - - - - - - - - - - - -
Presentation Transcript
Case presentation 18 frontiers 2011

Case Presentation #18Frontiers 2011

Lauren B. Smith, M.D.

Hematopathology


Clinical history 54 year old man
Clinical History: 54 year-old man

10/2001

Lymphocytosis

Splenomegaly

Adenopathy






Diagnosis
Diagnosis

  • B-cell non-Hodgkin lymphoma of small cells


Clinical history
Clinical History

10/2001

April 2009

Observation

Lymphocytosis

Splenomegaly

Adenopathy

Splenectomy

4625 g spleen


Spleen, April 2009 (H&E, magnification 20X)


Spleen, April 2009 (H&E, magnification 400X)



Cyclin D1

Spleen, April 2009 (magnification 20X)


Repeat Cyclin D1

Femoral lymph node biopsy October 2001 (100X magnification)


Clinical history1
Clinical History

10/2001

April 2009

July 2009

Observation

7.5 years

Lymphocytosis

Splenomegaly

Adenopathy

Dyspnea/

Worsening

Lymphadenopathy

ARF, tumor lysis

Splenectomy

4625 g spleen



IGH/CCND1 = 24500/

CCND1

IGH

IGH/CCND1

Peripheral blood FISH (8/2009): nuc ish(IGHx3), (CCND1x3), (IGH con CCND1x2) [72/200]


B-cell (IGH) Gene rearrangement Analysis by PCR on paraffin blocks

Spleen sample (April 2009)

Femoral lymph node (October 2001)

clonal identity (122 base pair amplicon)


Clinical history2
Clinical History blocks

10/2001

April 2009

July 2009

Dec 2009

Observation

7.5 years

Lymphocytosis

Splenomegaly

Adenopathy

Dyspnea/

Worsening

Lymphadenopathy

ARF, tumor lysis

Splenectomy

4625 g spleen

CNS dz


Diagnosis1
Diagnosis blocks

  • Indolent mantle cell lymphoma (2001)

  • Pleomorphic mantle cell lymphoma (2009)


Mcl variants
MCL Variants blocks

  • Blastoid

    • Fine chromatin

    • High mitotic rate (20-30/10 hpf)

    • High proliferative fraction (Ki-67)

  • Pleomorphic

    • Large with oval to irregular nuclear contours

    • Prominent nucleoli, pale cytoplasm


Indolent mantle cell
Indolent Mantle Cell blocks

  • Typical MCL

    • Widespread disease at diagnosis

    • Survival 3-5 years with chemotherapy

  • Indolent MCL

    • Bone marrow/spleen/GI-based disease

    • Usually no lymphadenopathy

    • 7-10 year survival (often untreated)


Indolent populations
Indolent Populations blocks

  • PB/Bone marrow only

    • Ondrejka et al (2011)

  • PB/Bone marrow, spleen, GI tract

    • Orchard et al (2003)

    • Fernandez et al (2010)


Gene expression profiling
Gene Expression Profiling blocks

  • SOX11 (2p25.3)

    • Involved in neuronal development

    • Possibly a tumor-suppressor gene in MCL

    • Immunohistochemistry

      • Expressed in 78-93% of MCL cases

      • Expressed in many cyclin D1 negative cases

      • Expressed in B/T-LBL, Burkitt, T-PLL, Hairy cell

      • Negative expression in many indolent cases

      • Not present in CLL/SLL, marginal zone, FL, Myeloma with t(11;14)



Take home points mcl
Take Home Points: MCL blocks

  • Clinical and morphologic variants exist

  • Recommend Cyclin D1 on CD5 negative splenectomy specimens

  • SOX11 may be helpful

  • Further study is needed


Acknowledgements blocksCase Submission for the XVth Meeting of the European Association for Haematopathology – Lymphoma Workshop

M. Chiselite, B. Schnitzer, D. Roulston and LB. Smith

University of Michigan Department of Pathology, Ann Arbor, MI, USA


References
References blocks

  • Bosch F, Lopez-Guillermo A, Campo E, Ribera JM, Conde E, Piris MA, et al. Mantle cell lymphoma: Presenting features, response to therapy, and prognostic factors. Cancer 1998;101(2)302-310.

  • Fernandez V, Salamero O, Espinet B, Sole F, Royo C, Navarro A, et al. Genomic and gene expression profiling defines indolent forms of mantle cell lymphoma. Cancer Res 2010;70(4):1408-18.

  • Ondrejka SL, Lai R, Smith SD & His ED. Indolent mantle cell leukemia: a clinicopathological variant characterized by isolated lymphocytosis, interstitial bone marrow involvement, kappa light chain restriction, and good prognosis. Hematologica 2011;96(8): 1121-27.

  • Orchard J, Garand R, Davis Z, Babbage G, Sahota S, Matutes E, et al. A subset of t(11;14) lymphoma with mantle cell features displays mutated IgVH genes and includes patient with good prognosis, nonnodal disease. Blood. 2003;101(12):4975-81.

  • Wang X, Asplund AC, Porwit A, Flygare J, Smith CI, Christensson B, et al. The subcellular SOX11 distribution pattern identifies subsets of mantle cell lymphoma: Correlation to overall survival. Br J Hematol 2008;143(2):248-52.

  • Xu Wei & Jian-Yong Li. SOX11 expression in mantle cell lymphoma. Leuk & Lymph 2010;51(11): 1962-67.


ad