Skip to main content

Advertisement

SpringerLink
Log in

Leukemic mantle cell lymphoma and chronic lymphocytic leukemia: a rare composite lymphoma and literature review

  • Case Report
  • Published:
Journal of Hematopathology Aims and scope Submit manuscript

Abstract

Composite lymphomas are rare entities that either represent a collision of two separate lymphomas, or one lymphoma that expresses two distinct phenotypes. Mantle cell lymphoma has been reported in some composite lymphomas, but when combined with chronic lymphocytic leukemia, it can be difficult to recognize because of the phenotypic overlap of these two entities. We report a case of a 75-year-old male with a slowly progressing lymphocytosis, but who was otherwise asymptomatic. Flow cytometry revealed two different populations, one showing bright Lambda positivity and CD5(+), while the other was positive for both lambda and kappa and was CD5(+). Subsequently, cytogenetics revealed two different populations, one with a trisomy 12 and the other with a t(11;14). Additionally, immunohistochemistry on a bone marrow biopsy showed SOX-11 to be negative and cyclin D1 to be positive on scattered lymphocytes. This was consistent with a composite lymphoma of leukemic mantle cell lymphoma and chronic lymphocytic lymphoma.

This is a preview of subscription content, log in via an institution to check access.

Access this article

Log in via an institution

Price excludes VAT (USA)
Tax calculation will be finalised during checkout.

Instant access to the full article PDF.

Institutional subscriptions

Fig. 1
Fig. 2
Fig. 3

Similar content being viewed by others

References

  1. Custer R (1954) Pitfalls in the diagnosis of lymphoma and leukemia from the pathologist’s point of view. Second National Cancer Conference; New York:554–557

  2. Küppers R, Dührsen U, Hansmann ML (2014) Pathogenesis, diagnosis, and treatment of composite lymphomas. Lancet Oncol 15(10):e435–e446

    Article  PubMed  CAS  Google Scholar 

  3. Sun Y, Wang SA, Sun T (2017) Composite mantle cell lymphoma and chronic lymphocytic leukemia/small lymphocytic lymphoma. Cytometry B Clin Cytom. https://doi.org/10.1002/cyto.b.21512. [Epub ahead of print]

  4. Schliemann I, Oschlies I, Nagel I, Maria Murga Penas E, Siebert R, Sander B (2016) The t(11;14)(q13;q32)/CCND1-IGH translocation is a recurrent secondary genetic aberration in relapsed chronic lymphocytic leukemia. Leuk Lymphoma 57(11):2672–2676

    Article  PubMed  Google Scholar 

  5. Ondrejka SL, Lai R, Smith SD, Hsi ED (2011) Indolent mantle cell leukemia: a clinicopathological variant characterized by isolated lymphocytosis, interstitial bone marrow involvement, kappa light chain restriction, and good prognosis. Haematologica 96(8):1121–1127

    Article  PubMed  PubMed Central  Google Scholar 

  6. Kern W, Bacher U, Schnittger S, Dicker F, Alpermann T, Haferlach T, Haferlach C (2014) Flow cytometric identification of 76 patients with biclonal disease among 5523 patients with chronic lymphocytic leukaemia (B-CLL) and its genetic characterization. Br J Haematol 164(4):565–569

    Article  PubMed  CAS  Google Scholar 

  7. Hallek M (2017) Chronic lymphocytic leukemia: 2017 update on diagnosis, risk stratification, and treatment. Am J Hematol 92(9):946–965

    Article  PubMed  CAS  Google Scholar 

  8. Sander B, Quintanilla-Martinez L, Ott G, Xerri L, Kuzu I, Chan JK, Swerdlow SH, Campo E (2016) Mantle cell lymphoma—a spectrum from indolent to aggressive disease. Virchows Arch 468(3):245–257

    Article  PubMed  CAS  Google Scholar 

  9. Hu Z, Sun Y, Schlette EJ, Tang G, Li S, Xu J, Yin CC, Young KH, Patel KP, Miranda RN, Goswami M, Wang M, Jorgensen JL, Medeiros LJ, Wang SA (2017) CD200 expression in mantle cell lymphoma identifies a unique subgroup of patients with frequent IGHV mutations, absence of SOX11 expression, and an indolent clinical course. Mod Pathol. https://doi.org/10.1038/modpathol.2017.135. [Epub ahead of print]

  10. Hoeller S, Zhou Y, Kanagal-Shamanna R, Xu-Monette ZY, Hoehn D, Bihl M, Swerdlow SH, Rosenwald A, Ott G, Said J, Dunphy CH, Bueso-Ramos CE, Lin P, Wang M, Miranda RN, Tzankov A, Medeiros LJ, Young KH (2013) Composite mantle cell lymphoma and chronic lymphocytic leukemia/small lymphocytic lymphoma: a clinicopathologic and molecular study. Hum Pathol 44(1):110–121

    Article  PubMed  CAS  Google Scholar 

  11. Fend F, Quintanilla-Martinez L, Kumar S, Beaty MW, Blum L, Sorbara L, Jaffe ES, Raffeld M (1999) Composite low grade B-cell lymphomas with two immunophenotypically distinct cell populations are true biclonal lymphomas. A molecular analysis using laser capture microdissection. Am J Pathol 154:1857–1866

    Article  PubMed  PubMed Central  CAS  Google Scholar 

  12. Kourelis TV, Kahl BS, Benn P, Delach JA, Bilgrami SF (2011) Treatment of synchronous mantle cell lymphoma and small lymphocytic lymphoma with bendamustine and rituximab. Acta Haematol 126:40–43

    Article  PubMed  CAS  Google Scholar 

  13. Nygren L, Baumgartner Wennerholm S, Klimkowska M, Christensson B, Kimby E, Sander B (2012) Prognostic role of SOX11 in a population-based cohort of mantle cell lymphoma. Blood 119(18):4215–4223

    Article  PubMed  CAS  Google Scholar 

  14. Wang M, Rule S, Zinzani PL, Goy A, Casasnovas O, Smith SD, Damaj G, Doorduijn J, Lamy T, Morschhauser F, Panizo C, Shah B, Davies A, Eek R, Dupuis J, Jacobsen E, Kater AP, Le Gouill S, Oberic L, Robak T, Covey T, Dua R, Hamdy A, Huang X, Izumi R, Patel P, Rothbaum W, Slatter JG, Jurczak W (2017) Acalabrutinib in relapsed or refractory mantle cell lymphoma (ACE-LY-004): a single-arm, multicentre, phase 2 trial. Lancet. pii: S0140-6736(17)33108-2. https://doi.org/10.1016/S0140-6736(17)33108-2. [Epub ahead of print]

  15. Wang ML, Rule S, Martin P, Goy A, Auer R, Kahl BS, Jurczak W, Advani RH, Romaguera JE, Williams ME, Barrientos JC, Chmielowska E, Radford J, Stilgenbauer S, Dreyling M, Jedrzejczak WW, Johnson P, Spurgeon SE, Li L, Zhang L, Newberry K, Ou Z, Cheng N, Fang B, McGreivy J, Clow F, Buggy JJ, Chang BY, Beaupre DM, Kunkel LA, Blum KA (2013) Targeting BTK with ibrutinib in relapsed or refractory mantle-cell lymphoma. N Engl J Med 369(6):507–516

    Article  PubMed  PubMed Central  CAS  Google Scholar 

Download references

Author information

Authors and Affiliations

  1. Department of Pathology, New York University Medical Center, 530 First Avenue, New York, NY, 10010, USA

    Etan Marks, Cynthia Liu & Arnaldo Arbini

  2. Hematology Division, Department of Medicine, New York University Medical Center, 550 First Avenue, New York, NY, 10010, USA

    Bruce Raphael

Authors
  1. Etan Marks
    View author publications

    You can also search for this author in PubMed Google Scholar

  2. Cynthia Liu
    View author publications

    You can also search for this author in PubMed Google Scholar

  3. Bruce Raphael
    View author publications

    You can also search for this author in PubMed Google Scholar

  4. Arnaldo Arbini
    View author publications

    You can also search for this author in PubMed Google Scholar

Corresponding author

Correspondence to Etan Marks.

Ethics declarations

Conflict of interest

The authors declare that they have no conflict of interest.

Informed consent from patient

Informed consent was received from the patient for this paper to be published.

Additional information

Ethical responsibilities of authors section:

Virchows Archiv conforms to the ICMJE recommendation for qualification of authorship. The ICMJE recommends that authorship be based on the following four criteria:

• Substantial contributions to the conception or design of the work; or the acquisition, analysis, or interpretation of data for the work; AND

• Drafting the work or revising it critically for important intellectual content; AND

• Final approval of the version to be published; AND

• Agreement to be accountable for all aspects of the work in ensuring that questions related to the accuracy or integrity of any part of the work are appropriately investigated and resolved.

All listed authors fit these criteria.

Rights and permissions

Reprints and permissions

About this article

Check for updates. Verify currency and authenticity via CrossMark

Cite this article

Marks, E., Liu, C., Raphael, B. et al. Leukemic mantle cell lymphoma and chronic lymphocytic leukemia: a rare composite lymphoma and literature review. J Hematopathol 11, 57–62 (2018). https://doi.org/10.1007/s12308-018-0321-7

Download citation

  • Received:

  • Accepted:

  • Published:

  • Issue Date:

  • DOI: https://doi.org/10.1007/s12308-018-0321-7

Keywords

Search

Navigation