Anaplastic Large Cell Lymphoma (ALCL) is a type of lymphoma (abnormal lymphocyte) affecting mainly t-cells. It is generally treated as an aggressive lymphoma with intent to cure.
NOTE: We urge patients diagnosed with ALCL to seek guidance (if feasible) at a center that specializes in cooperative research. See for a list of centers.
"The definition of anaplastic large cell lymphoma (ALCL) has evolved since its original description in 1985 by Stein and colleagues (1) as a lymphoma characterized by large anaplastic lymphoid cells with uniform, strong expression of CD30 and a tendency to grow cohesively and invade lymph node sinuses.
Subsequent immunophenotypic and genetic studies resulted in restriction of the diagnosis to cases of T-cell or null lineage, and recognition that primary cutaneous and systemic types were clinically and immunophenotypically* distinctive.
The current World Health Organization (WHO) (2) classification distinguishes systemic ALCL from primary cutaneous ALCL (cut-ALCL)." http://bloodjournal.hematologylibrary.org
* immunophenotypically distinctive means differing in stage of maturation - based on what is expressed on the abnormal lymphoid cells - as it relates to the cell of origin - the corresponding normal lymphoid cells.
Based on clinical manifestations, CD30+ ALCL that presents in the skin can be subdivided mainly into
1) primary cutaneous (skin) form without extracutaneous involvement at presentation, or
2) systemic form with secondary skin involvement at presentation." 1
"ALK-negative ALCL is defined in the WHO classification as a lymphoma that is morphologically within the spectrum of ALK-pos ALCL, with strong and uniform expression of CD30, but lacking ALK protein expression" Source: bloodjournal.hematologylibrary.org
TREATMENT - Standard of care depends on the following:
- cutaneous or systemic presentation,
- if cells are ALK -/+), and clinical circumstances
BEST PRACTICE (Standard of Care): NCCN.org PDF (pages 69-71) requires one-time free registration and login.
Notable new approved treatment agent:
Brentuximab Vedotin (SGN-35) in Patients With Relapsed or Refractory Systemic Anaplastic Large-Cell Lymphoma: Results of a Phase II Study http://jco.ascopubs.org/content/30/18/2190.short
Conclusion Brentuximab vedotin induced objective responses in the majority of patients and CRs in more than half of patients with recurrent systemic ALCL. Targeted therapy with this CD30-directed antibody-drug conjugate may be an effective treatment for relapsed or refractory systemic ALCL and warrants further studies in front-line therapy.
About Cutaneous CD30+ (Ki-1) Anaplastic Large-Cell Lymphoma e-medicine
About ALCL Wikipedia.org
Anaplastic large-cell lymphoma: clinical and prognostic evaluation of 90 adult patients. J Clin Oncol. 1996 Mar;14(3):955-62. PMID: 8622045 PubMed
Neutrophil-Rich Ki-1-Positive Anaplastic Large Cell Lymphoma Medscape (free login req. (from American Journal of Clinical Pathology )
Anaplastic large cell lymphoma, T/null cell, primary cutaneous or systemic type
Related PubMed Abstracts
Adult Anaplastic large cell lymphoma Related PubMed Abstracts
Childhood Anaplastic large cell lymphoma Related PubMed Abstracts
ASCO 2003 - SGN-30 is a genetically engineered antibody in clinical trials for the treatment of patients with CD30+ hematologic malignancies such as Hodgkin's disease, anaplastic large cell lymphoma and other types of lymphomas. ASCO
A population-based study of pediatric anaplastic large cell lymphoma. Cancer. 2002 Mar 15;94(6):1830-5. PMID: 11920547 PubMed
Distinct effects of CD30 and Fas signaling in cutaneous anaplastic lymphomas: a possible mechanism for disease progression. J Invest Dermatol. 2000 Dec;115(6):1034-40. PMID: 11121138 PubMed
Trofosfamide as salvage therapy for anaplastic large cell lymphoma relapsing after high-dose chemotherapy. Leuk Lymphoma. 2002 Dec;43(12):2351-3.
PMID: 12613523 PubMed
Case study: "This therapy resulted in a complete remission, still ongoing, 27 months after termination of intravenous cytotoxic therapy and 16 months after withdrawal of trofosfamide. Thus, in this particular case, trofosfamide turned out to be an unexpectedly effective salvage therapy for an otherwise very aggressive relapsing ALCL."
Neutrophil-Rich Anaplastic Large Cell Lymphoma of T-Cell Lineage: A Report of Two Cases Arising in HIV-Positive Patients Medscape free login req.
Anaplastic Large-Cell Lymphoma, T-/Null-Cell Type Eric Jacobsen, The Oncologist, July 2006 http://bit.ly/aKYe7y
ALK-negative anaplastic large-cell lymphoma (ALCL) is different from both ALK-positive ALCL and peripheral T-cell lymphoma, not otherwise specified http://bloodjournal.hematologylibrary.org
ALK-positive ALCL patients had a superior outcome compared to those with ALK-negative ALCL
Primary cutaneous ALCL patients had a very favorable 5 y OS (90%), but with a propensity to relapse (5 y FFS 55%).
ALK-negative ALCL should continue to be separated from both ALK-positive ALCL and PTCL-NOS.
Although the prognosis of ALK negative ALCL appears to be better than PTCL-NOS, it is still unsatisfactory and better therapies are needed. Primary cutaneous ALCL is associated with an indolent course.
Prognostic Significance of Anaplastic Lymphoma Kinase (ALK) Protein Expression in Adults With Anaplastic Large Cell Lymphoma bloodjournal.hematologylibrary.org/
Seattle Genetics and Millennium Announce Positive Top-Line Brentuximab Vedotin (SGN-35) Data from Phase II Trial in Relapsed or Refractory ALCL Media report
"Based on overall response rates of 86 percent in ALCL and 75 percent in HL with single-agent brentuximab vedotin, we intend to discuss regulatory next steps with the U.S. Food and Drug Administration (FDA) later this year with the goal of including both indications in our Biologics License Application (BLA) submission planned for the first half of 2011," said Clay B. Siegall, Ph.D., President and Chief Executive Officer of Seattle Genetics. "These remarkable data in relapsed or refractory patients highlight the broader opportunity for brentuximab vedotin in CD30-positive hematologic malignancies and reinforce our ongoing and planned clinical development activities. We believe that brentuximab vedotin represents the next step in the evolution of targeted therapy and could be the first in a new class of advanced generation ADCs."
Long-term follow-up of patients with peripheral T-cell lymphomas treated up-front with high-dose chemotherapy followed by autologous stem cell transplantation.
Leukemia. 2006 Sep;20(9):1533-8. Epub 2006 Jul 27. PMID: 16871285
... our findings indicate (1) up-front high-dose therapy and ASCT are feasible, but could induce a high rate of long-term CR only in patients with ALK-positive ALCL and (2) the achievement of CR before auto-grafting is a strong predictor of better survival."
Allogeneic haematopoietic stem cell transplantation in relapsed or refractory anaplastic large cell lymphoma of children and adolescents--a Berlin-Frankfurt-Munster group report.
Br J Haematol. 2006 Apr;133(2):176-82. PMID: 16611309
Autologous stem cell transplantation for anaplastic large-cell lymphomas: results of a prospective trial. Br J Haematol. 2000 Jun;109(4):736-42. PMID: 10929023
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 http://www.nature.com/bmt/journal/v33/n6/full/1704392a.html