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Types > T-cell > Anaplastic Large Cell Lymphoma (ALCL)

Last update: 05/18/2010

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Childhood NHL - Anaplastic Large Cell

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"Cutaneous CD30+ (Ki-1) anaplastic large-cell lymphoma (cutaneous CD30+ ALCL) is clinically and pathologically heterogeneous (varying), leading to some difficulty in its diagnosis and classification. 

Expression of CD30 (an activation marker for B or T cells) in more than 75% of neoplastic cells characterizes this group of disorders.

Based on clinical manifestations, cutaneous CD30+ ALCL 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

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TREATMENT - Standard of care depends on

cutaneous or systemic presentation,
 
if cells are ALK -/+), and clinical circumstances
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BEST PRACTICE (Standard of Care):  NCCN.org  PDF (pages 69-71) 
requires one-time free registration and login.

Notable new investigational treatment agent:

SGN-30 (Anti-CD30 mAb) Has a Single-Agent Response Rate of 21% in Patients with Refractory or Recurrent Systemic Anaplastic Large Cell Lymphoma (ALCL). Session Type: Poster Session, Board #896-II  ASH 2006

This phase II study represents one of the largest prospectively designed trials in relapsed/refractory ALCL and demonstrates good tolerability and clinically meaningful antitumor activity of SGN-30, especially in ALK negative patients who have a particularly poor prognosis.


Resources 

  1. About Cutaneous CD30+ (Ki-1) Anaplastic Large-Cell Lymphoma  e-medicine 
  2. About  Wikipedia.org
  3. Anaplastic large-cell lymphoma: clinical and prognostic evaluation of 90 adult patients.
    J Clin Oncol. 1996 Mar;14(3):955-62. PMID: 8622045  PubMed
  4. Neutrophil-Rich Ki-1-Positive Anaplastic Large Cell Lymphoma Medscape (free login req. (from American Journal of Clinical Pathology )
  5. Anaplastic large cell lymphoma, T/null cell, primary cutaneous or systemic  type
    Related PubMed Abstracts
  6. Adult Anaplastic large cell lymphoma  Related PubMed Abstracts
  7. Childhood Anaplastic large cell lymphoma  Related PubMed Abstracts
  8. 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
  9. A population-based study of pediatric anaplastic large cell lymphoma.
    Cancer. 2002 Mar 15;94(6):1830-5. PMID: 11920547  PubMed
  10. 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 
  11. 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." 
  12. Neutrophil-Rich Anaplastic Large Cell Lymphoma of T-Cell Lineage: A Report of Two Cases Arising in HIV-Positive Patients Medscape  free login req.
  13. Anaplastic Large-Cell Lymphoma, T-/Null-Cell Type
    Eric Jacobsen, The Oncologist, July 2006 http://bit.ly/aKYe7y
  14. 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.
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PubMed Queries on Anaplastic Large Cell Lymphoma

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 Diagnosis | Review | Therapies | Prognosis
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Clinical Trials

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Clinical trials in ClinicalTrials.gov for Anaplastic Large Cell
Newly diagnosed or  previously untreated
  AND Recurrent
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ClinicalTrials.gov studies for T-cell lymphomas
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Research News

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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."
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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
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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
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ALK anaplastic large-cell lymphoma http://bloodjournal.hematologylibrary.org/cgi/content/full/111/12/5496 
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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
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