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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 ).

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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

slide12

Cyclin D1

Spleen, April 2009 (magnification 20X)

slide13

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

slide16

IGH/CCND1

CCND1

IGH

IGH/CCND1

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

slide17

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

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
  • Indolent mantle cell lymphoma (2001)
  • Pleomorphic mantle cell lymphoma (2009)
mcl variants
MCL Variants
  • 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
  • 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
  • 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
  • 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
  • Clinical and morphologic variants exist
  • Recommend Cyclin D1 on CD5 negative splenectomy specimens
  • SOX11 may be helpful
  • Further study is needed
slide26

AcknowledgementsCase 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
  • 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.
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