Skip to main content
SpringerLink
Log in

Anaplastic Large Cell Lymphoma

  • Chapter
  • First Online:
Rare Lymphomas

Part of the book series: Hematologic Malignancies ((HEMATOLOGIC))

  • 1105 Accesses

Abstract

Anaplastic large cell lymphoma (ALCL) is a rare lymphoid malignancy that is characterized by the expression of CD30 cell surface receptor and by a chromosomal translocation that results in aberrant constitutive activation of the anaplastic large cell lymphoma kinase (ALK). Like many other types of lymphoma, no major advances in the treatment of ALCL have been made for almost three decades. This is mainly because the relatively high cure rate and the rarity of the disease. Current frontline multi-agent chemotherapy regimens cure approximately 50 % of the patients, and patients with ALK-positive lymphoma usually have a better prognosis. More recently, advances in the molecular biology of ALCL have led to the development of new targeted agents. Brentuximab vedotin which targets CD30 receptor induces 85 % response rate in patients with relapsed ALCL and was recently approved by the US Food and Drug Administration for this disease. Crizotinib, which targets the ALK kinase and has shown promising results in preclinical and a limited clinical setting, is currently being evaluated in phase II clinical studies. Future directions will aim at changing current treatment strategies by incorporating these two new agents in frontline regimens.

Pathology: Scott Rodig and Jan Delabie

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

Access this chapter

Log in via an institution
Chapter
USD 29.95
Price excludes VAT (USA)
  • Available as PDF
  • Read on any device
  • Instant download
  • Own it forever
eBook
USD 84.99
Price excludes VAT (USA)
  • Available as EPUB and PDF
  • Read on any device
  • Instant download
  • Own it forever
Softcover Book
USD 159.99
Price excludes VAT (USA)
  • Compact, lightweight edition
  • Dispatched in 3 to 5 business days
  • Free shipping worldwide - see info
Hardcover Book
USD 109.99
Price excludes VAT (USA)
  • Durable hardcover edition
  • Dispatched in 3 to 5 business days
  • Free shipping worldwide - see info

Tax calculation will be finalised at checkout

Purchases are for personal use only

Institutional subscriptions

References

  • Abouyabis AN, Shenoy PJ, Sinha R, Flowers CR, Lechowicz MJ (2011) A systematic review and meta-analysis of front-line anthracycline-based chemotherapy regimens for peripheral T-cell lymphoma. ISRN Hematol 2011:623924

    Article  PubMed Central  PubMed  Google Scholar 

  • Ansell SM, Byrd JC, Horwitz SB et al (2003) Phase I/II study of a fully human anti-CD30 monoclonal antibody (MDX-060) in Hodgkin's disease (HD) and anaplastic large cell lymphoma (ALCL). Blood 102:632 (abstract)

    Google Scholar 

  • Beitinjaneh A, Saliba RM, Okoroji GJ et al (2011) Autologous and allogeneic stem cell transplantation for T-cell lymphoma: the MD Anderson Cancer Center experience. Blood 118:Abstract 4118

    Google Scholar 

  • Benharroch D et al (1998) ALK-Positive lymphoma: a single disease with a broad spectrum of morphology. Blood 91(6):2076–2084

    CAS  PubMed  Google Scholar 

  • Brugieres I, Deley MC, Pacquement H et al (1998) CD30 (+) anaplastic large cell lymphoma in children: analysis of 82 patients enrolled in two consecutive studies of the French Society of Pediatric Oncology. Blood 92:3591–3598

    CAS  PubMed  Google Scholar 

  • Chiarle R et al (2003) NPM-ALK transgenic mice spontaneously develop T-cell lymphomas and plasma cell tumors. Blood 101(5):1919–1927

    Article  CAS  PubMed  Google Scholar 

  • Chott A et al (1990) Ki-1-positive large cell lymphoma. A clinicopathologic study of 41 cases. Am J Surg Pathol 14(5):439–448

    Article  CAS  PubMed  Google Scholar 

  • Corradini P, Dodero A, Zallio F et al (2004) Graft-versus-lymphoma effect in relapsed peripheral T-cell non-Hodgkin’s lymphomas after reduced-intensity conditioning followed by allogeneic transplantation of hematopoietic cells. J Clin Oncol 22:2172–2176

    Article  PubMed  Google Scholar 

  • Costes-Martineau V, Delfour C, Obled S et al (2002) Anaplastic lymphoma kinase (ALK) protein expressing lymphoma after liver transplantation: case report and literature review. J Clin Pathol 55:868–871

    Article  CAS  PubMed  Google Scholar 

  • d’Amore F, Relander T, Lauritzsen GF et al (2012) Up-front autologous stem-cell transplantation in peripheral T-cell lymphoma: NLG-T-01. J Clin Oncol 30:3093–3099

    Article  PubMed  Google Scholar 

  • Dearden CE, Johnson R, Pettengell R et al (2011) British Committee for Standards in Haematology. Guidelines for the management of mature T-cell and NK-cell neoplasms (excluding cutaneous T-cell lymphoma). Br J Haematol 153:451–485

    Article  PubMed  Google Scholar 

  • Delsol G, Jaffe ES, Falini B et al (2008) Anaplastic large cell lymphoma (ALCL), ALK-positive. In: Swerdlow S, Campo E, Harris NL (eds) WHO classification of tumors of hematopoietic and lymphoid tissues, 4th edn. IARC, Lyon, pp 312–316

    Google Scholar 

  • Falini B et al (1999) ALK+ lymphoma: clinico-pathological findings and outcome. Blood 93(8):2697–2706

    CAS  PubMed  Google Scholar 

  • Forero-Torres A, Leonard JP, Younes A et al (2009) A Phase II study of SGN-30 (anti-CD30 mAb) in Hodgkin lymphoma or systemic anaplastic large cell lymphoma. Br J Haematol 146:171–179

    Article  CAS  PubMed  Google Scholar 

  • Foss HD et al (1996) Anaplastic large-cell lymphomas of T-cell and null-cell phenotype express cytotoxic molecules. Blood 88(10):4005–4011

    CAS  PubMed  Google Scholar 

  • Fraga M, Brousset P, Schlaifer D et al (1995) Bone marrow involvement in anaplastic large cell lymphoma: immunohistochemical detection of minimal disease and its prognostic significance. Am J Clin Pathol 103:82–89

    CAS  PubMed  Google Scholar 

  • Gabarre J, Raphael M, Lepage E et al (2001) Human immunodeficiency virus-related lymphoma: relation between clinical features and histologic subtypes. Am J Med 111:704–711

    Article  CAS  PubMed  Google Scholar 

  • Gallamini A, Stelitano C, Calvi R et al (2004) Peripheral T-cell lymphoma unspecified (PTCL-U): a new prognostic model from a retrospective multicentric clinical study. Blood 103:2474–2479

    Article  CAS  PubMed  Google Scholar 

  • Gambacorti-Passerini C, Messa C, Pogliani EM (2011) Crizotinib in anaplastic large-cell lymphoma. N Engl J Med 364:775–776

    Article  PubMed  Google Scholar 

  • Gascoyne RD et al (1999) Prognostic significance of anaplastic lymphoma kinase. Blood 93(11):3913–3921

    CAS  PubMed  Google Scholar 

  • Harris NL, Jaffe ES, Stein H et al (1994) A revised European-American classification of lymphoid neoplasms: a proposal from the International Lymphoma Study Group. Blood 84:1361–1392

    CAS  PubMed  Google Scholar 

  • Hosing C, Champlin RE (2011) Stem-cell transplantation in T-cell non-Hodgkin’s lymphomas. Ann Oncol 22:1471–1477

    Article  CAS  PubMed  Google Scholar 

  • Jagasia M, Morgan D, Goodman S et al (2004) Histology impacts the outcome of peripheral T-cell lymphomas after high dose chemotherapy and stem cell transplant. Leuk Lymphoma 45:2261–2267

    Article  CAS  PubMed  Google Scholar 

  • Kadin ME, Morris SW (1998) The t(2;5) in human lymphomas. Leuk Lymphoma 29:249–256

    Article  CAS  PubMed  Google Scholar 

  • Kalinova M, Krskova L, Brizova H, Kabickova E, Kepak T, Kodet R (2008) Quantitative PCR detection of NPM/ALK fusion gene and CD30 gene expression in patients with anaplastic large cell lymphoma—residual disease monitoring and a correlation with the disease status. Leuk Res 32:25–32

    Article  CAS  PubMed  Google Scholar 

  • Katz J, Janik JE, Younes A (2011) Brentuximab Vedotin (SGN-35). Clin Cancer Res 17:6428–6436

    Article  CAS  PubMed  Google Scholar 

  • Kim B, Roth C, Young VL et al (2011) Anaplastic large cell lymphoma and breast implants: results from a structured expert consultation process. Plast Reconstr Surg 128:629–639

    Article  CAS  PubMed  Google Scholar 

  • Kinney MC et al (1993) A small-cell-predominant variant of primary Ki-1 (CD30)+ T-Cell lymphoma. Am J Surg Pathol 17(9):859–868

    Article  CAS  PubMed  Google Scholar 

  • Kwong YL, Anderson BO, Advani R, Kim WS, Levine AM, Lim ST (2009) Management of T-cell and natural-killer-cell neoplasms in Asia: consensus statement from the Asian Oncology Summit 2009. Lancet Oncol 10:1093–1101

    Article  PubMed  Google Scholar 

  • Lazzeri D, Agostini T, Bocci G et al (2011) ALK-1-negative anaplastic large cell lymphoma associated with breast implants: a new clinical entity. Clin Breast Cancer 11:283–296

    Article  PubMed  Google Scholar 

  • Mason DY, Harris NL, Delsol G et al (2008) Anaplastic large cell lymphoma, ALK-negative. In: Swerdlow S, Campo E, Harris NL et al (eds) WHO classification of tumors of hematopoietic and lymphoid tissues, 4th edn. IARC, Lyon, pp 317–319

    Google Scholar 

  • Morris SW et al (1995) Fusion of a kinase gene, ALK, to a nucleolar protein gene, NPM, in non-Hodgkin’s lymphoma. Science 267:316–317

    Article  CAS  PubMed  Google Scholar 

  • Mussolin L, Pillon M, d’Amore ES et al (2005) Prevalence and clinical implications of bone marrow involvement in pediatric anaplastic large cell lymphoma. Leukemia 19:1643–1647

    Article  CAS  PubMed  Google Scholar 

  • National Comprehensive Cancer Network (NCCN) (2012) NCCN clinical practice guidelines in oncology: Non-Hodgkin lymphoma, Version 2.2012. http://www.nccn.org/professionals/physician_gls/pdf/nhl.pdf Accessed 14 Mar 2012

  • Ou SH (2011) Crizotinib: a novel and first-in-class multitargeted tyrosine kinase inhibitor for the treatment of anaplastic lymphoma kinase rearranged non-small cell lung cancer and beyond. Drug Des Devel Ther 5:471–485

    Article  PubMed Central  PubMed  Google Scholar 

  • Pileri SA et al (1997) Frequent expression of the p80 NPM-ALK chimeric fusion protein in anaplastic large-cell lymphoma, lympho-histiocytic type. Am J Pathol 150(4):1207–1211

    CAS  PubMed  Google Scholar 

  • Piva R, Agnelli L, Pellegrino E et al (2010) Gene expression profiling uncovers molecular classifiers for the recognition of anaplastic large-cell lymphoma within peripheral T-cell neoplasms. J Clin Oncol 28:1583–1590

    Article  CAS  PubMed  Google Scholar 

  • Popplewell L, Thomas SH, Huang Q et al (2011) Primary anaplastic large-cell lymphoma associated with breast implants. Leuk Lymphoma 52:1481–1487

    Article  PubMed Central  PubMed  Google Scholar 

  • Pro B, Advani R, Brice P et al (2012) Brentuximab vedotin (SGN-35) in patients with relapsed or refractory systemic anaplastic large-cell lymphoma: results of a phase II study. J Clin Oncol 30:2190–2196

    Article  PubMed  Google Scholar 

  • Ralfkiaer E, Willemze R, Paulli M, Kadin M (2008) Primary cutaneous CD30-positive T-cell lymphoproliferative disorders. In: Swerdlow S, Campo E, Harris NL et al (eds) WHO classification of tumors of hematopoietic and lymphoid tissues, 4th edn. IARC, Lyon, pp 300–301

    Google Scholar 

  • Reimer P, Rüdiger T, Geissinger E et al (2009) Autologous stem-cell transplantation as first-line therapy in peripheral T-cell lymphomas: results of a prospective multicenter study. J Clin Oncol 27:106–113

    Article  CAS  PubMed  Google Scholar 

  • Rizvi MA, Evens AM, Tallman MS, Nelson BP, Rosen ST (2006) T-cell non-Hodgkin lymphoma. Blood 107:1255–1264

    Article  CAS  PubMed  Google Scholar 

  • Salhany KE, Cousar JB, Greer JP et al (1988) Transformation of cutaneous T cell lymphoma to large cell lymphoma. A clinicopathologic and immunologic study. Am J Pathol 132:365–377

    Google Scholar 

  • Sandlund JT, Pui CH, Roberts WM et al (1994) Clinicopathologic features and treatment outcome of children with large- cell lymphoma and the t(2;5)(p23;q35). Blood 84:2467–2471

    CAS  PubMed  Google Scholar 

  • Savage KJ (2007) Peripheral T-cell lymphomas. Blood Rev 21:201–216

    Article  CAS  PubMed  Google Scholar 

  • Savage KJ, Harris NL, Vose JM et al (2008) ALK anaplastic large-cell lymphoma is clinically and immunophenotypically different from both ALK ALCL and peripheral T-cell lymphoma, not otherwise specified: report from the International Peripheral T-Cell Lymphoma Project. Blood 111:5496–5504

    Article  CAS  PubMed  Google Scholar 

  • Schmitz N, Trümper L, Ziepert M et al (2010) Treatment and prognosis of mature T-cell and NK-cell lymphoma: an analysis of patients with T-cell lymphoma treated in studies of the German High-Grade Non-Hodgkin Lymphoma Study Group. Blood 116:3418–3425

    Article  CAS  PubMed  Google Scholar 

  • Seidemann K, Tiemann M, Schrappe M et al (2001) Short-pulse B-non-Hodgkin lymphoma-type chemotherapy is efficacious treatment for pediatric anaplastic large cell lymphoma: a report of the Berlin-Frankfurt-Münster Group Trial NHL-BFM 90. Blood 97:3699–3706

    Article  CAS  PubMed  Google Scholar 

  • Shaw AT, Yasothan U, Kirkpatrick P (2011) Crizotinib. Nat Rev Drug Discov 10:897–898

    Article  CAS  PubMed  Google Scholar 

  • Stansfeld AG, Diebold J, Noel H et al (1988) Updated Kiel classification for lymphomas. Lancet 1:292–293

    Article  CAS  PubMed  Google Scholar 

  • Stein H, Mason DY, Gerdes J (1985) The expression of the Hodgkin’s disease associated antigen Ki-1 in reactive and neoplastic lymphoid tissue: evidence that Reed-Sternberg cells and histiocytic malignancies are derived from activated lymphoid cells. Blood 66:848–858

    CAS  PubMed  Google Scholar 

  • Stein H, Foss HD, Durkop H et al (2000) CD30+ anaplastic large cell lymphoma: a review of its histopathologic, genetic, and clinical features. Blood 96:3681–3695

    CAS  PubMed  Google Scholar 

  • Suzuki R, Kagami Y, Takeuchi K et al (2000) Prognostic significance of CD56 expression for ALK-positive and ALK-negative anaplastic large-cell lymphoma of T/null cell phenotype. Blood 96:2993–3000

    CAS  PubMed  Google Scholar 

  • Tilly H, Lepage E, Coiffier B et al (2003) Intensive conventional chemotherapy (ACVBP regimen) compared with standard CHOP for poor-prognosis aggressive non-Hodgkin lymphoma. Blood 102:4284–4289

    Article  CAS  PubMed  Google Scholar 

  • Tirelli U, Vaccher E, Zagonel V et al (1995) CD30 (ki-1)-positive anaplastic large-cell lymphomas in 13 patients with and 27 patients without human immunodeficiency virus infection: the first comparative clinicopathologic study from a single institution that also includes 80 patients with other human immunodeficiency virus-related sistemi lymphomas. J Clin Oncol 13:373–380

    CAS  PubMed  Google Scholar 

  • Trial watch: success for crizotinib in ALK-driven cancer (2010) Nat Rev Drug Discov 9:908

    Google Scholar 

  • Vose J, Armitage J, Weisenburger D (2008) International T-Cell Lymphoma Project. International peripheral T-cell and natural killer/T-cell lymphoma study: pathology findings and clinical outcomes. J Clin Oncol 26:4124–4130

    Article  PubMed  Google Scholar 

  • Went P et al (2006) Marker expression in peripheral T-cell lymphoma: a proposed clinical-pathologic prognostic score. J Clin Oncol 24(16):2472–2479

    Article  CAS  PubMed  Google Scholar 

  • Younes A (2011a) CD30-targeted antibody therapy. Curr Opin Oncol 23:587–593

    Article  CAS  PubMed  Google Scholar 

  • Younes A (2011b) Beyond chemotherapy: new agents for targeted treatment of lymphoma. Nat Rev Clin Oncol 8:85–96

    Article  CAS  PubMed Central  PubMed  Google Scholar 

  • Younes A, Bartlett NL, Leonard JP et al (2010) Brentuximab vedotin (SGN-35) for relapsed CD30-positive lymphomas. N Engl J Med 363:1812–1821

    Article  CAS  PubMed  Google Scholar 

  • Younes A, Yasothan U, Kirkpatrick P (2011) Brentuximab vedotin. Nat Rev Drug Discov 11:19–20

    Article  Google Scholar 

  • Zamkoff KW, Matulis MD, Metha AC et al (2004) High-dose therapy and autologous stem cell transplant does not result in long-term disease-free survival in patients with recurrent chemotherapy-sensitive ALK-negative anaplastic large-cell lymphoma. Bone Marrow Transplant 33:635–638

    Article  CAS  PubMed  Google Scholar 

Download references

Author information

Authors and Affiliations

  1. MD Anderson Cancer Center, The University of Texas, Houston, TX, USA

    Anas Younes

  2. Institute of Hematology “L. e A. Seràgnoli”, University of Bologna, Bologna, Italy

    Pier Luigi Zinzani

  3. Department of Pathology, Brigham and Women’s Hospital, Harvard Medical School, Boston, MA, USA

    Scott Rodig MD

  4. Department of Pathology, Oslo University Hospital, Oslo, Norway

    Jan Delabie MD

Authors
  1. Anas Younes
    View author publications

    You can also search for this author in PubMed Google Scholar

  2. Pier Luigi Zinzani
    View author publications

    You can also search for this author in PubMed Google Scholar

  3. Scott Rodig MD
    View author publications

    You can also search for this author in PubMed Google Scholar

  4. Jan Delabie MD
    View author publications

    You can also search for this author in PubMed Google Scholar

Corresponding author

Correspondence to Anas Younes .

Editor information

Editors and Affiliations

  1. Medizinischen Klinik und Poliklinik III, Universität München Klinikum Großhadern, München, Germany

    Martin Dreyling

  2. Division of Hematology/Oncology and Cancer Center, University of Virginia Health System, Charlottesville, Virginia, USA

    Michael E. Williams

Rights and permissions

Reprints and permissions

Copyright information

© 2014 Springer-Verlag Berlin Heidelberg

About this chapter

Cite this chapter

Younes, A., Zinzani, P.L., Rodig, S., Delabie, J. (2014). Anaplastic Large Cell Lymphoma. In: Dreyling, M., Williams, M. (eds) Rare Lymphomas. Hematologic Malignancies. Springer, Berlin, Heidelberg. https://doi.org/10.1007/978-3-642-39590-1_6

Download citation

  • DOI: https://doi.org/10.1007/978-3-642-39590-1_6

  • Published:

  • Publisher Name: Springer, Berlin, Heidelberg

  • Print ISBN: 978-3-642-39589-5

  • Online ISBN: 978-3-642-39590-1

  • eBook Packages: MedicineMedicine (R0)

Publish with us

Policies and ethics

Search

Navigation