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Anaplastic Large Cell
(Ki-1 / CD-30) Lymphoma

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Anaplastic large cell lymphoma (ALCL) can present itself in two forms: it can be systemic (throughout the body) in children or young adults or cutaneous (in/on the skin).   

Disease limited to the skin is quite slow growing (indolent). ALCL primary cutaneous type, manifests as a solitary nodule or ulcerating tumor in patients without a history of or concurrent Mycosis Fungoides (MF) or lymphomatoid papulosis (LyP) and no evidence of disease outside the skin.  Lymph nodes in the same region are involved 25% of the time.

The disease tests positive for the CD30 antigen.  Staging is required per other non-Hodgkin lymphomas. Patients may experience spontaneous remissions with relapses. If no spontaneous remission occurs, radiation and/or surgical excision are preferable. Chemotherapy is reserved for patients who have generalized lesions - low dose methotrexate has been shown to be an effective treatment.  Cutaneous ALCL in patients with a prior history of MF or LyP has a poorer prognosis. When ALCL secondarily involves the skin from nodes, the prognosis is worse. Primary cutaneous-type ALCL does not appear to have the gene translocation t(2;5).
 

Systemic ALCL can involve lymph nodes and extranodal sites acting aggressively but responds to chemotherapy used to treat other large cell lymphomas.  The systemic form is associated with a t(2;5) chromosomal abnormality, leading to the production of the  
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anaplastic lymphoma kinase (ALK) protein.  This so-called "classic" ALCL is most common in children and adolescents. 

Both types of ALCL can be from T-cell lymphocytes or cell type unknown (null).

Further Reading / In-Depth

Non-Hodgkin's Lymphomas, Peter M. Mauch (Editor), James O. Armitage (Editor), et al., 2004.
  • Section IV: Pathology, Biology, Clinical Evaluation, and Treatment Section
    • Chapter 25: Anaplastic Large Cell Lymphoma
    • Chapter 21: CD30+ Cutaneous Lymphoproliferative Disease (Anaplastic Large-Cell Lymphoma)

Excellent but Technical: An Illustrated Guide to Skin Lymphomas, Cerroni, Gatter, Kerl, 2nd. Ed, 2005

  • Chapter 4: CD30+ Cutaneous Lymphoproliferative Disorders
    • Anaplastic Large T-Cell Lymphoma, pp 52-58

Web Information

Mailing Lists

  • ALCL-Kids is a support group and information sharing discussion group for parents of children with Anaplastic Large Cell Lymphoma.
    •
  • skinlymphoma-notMFnotLyP is a group for people Large Cell Lymphoma of the skin. This disease is also called Cutaneous Anaplastic Large Cell Lymphoma, Ki-1 lymphoma of the skin, and Primary Cutaneous CD30+ lymphoma.  Of the skin lymphomas, it is second most common after Mycosis Fungoides.
  • More Mailing Lists

Sources

Non-Hodgkin's Lymphoma Information Pages:

    Non-Hodgkin's Lymphomas
    Non-Hodgkin's Lymphoma: Diagnosis
    Non-Hodgkin's Lymphoma: Aggressive Lymphomas
    Non-Hodgkin's Lymphoma: Indolent Lymphomas
    Non-Hodgkin's Lymphoma: Treatment
    Non-Hodgkin's Lymphoma: Resources
    Bone Marrow and Stem Cell Transplants

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This page is a work in progress - if you have more complete information, references, or other information please contact the author.  The author is not in the medical field and does not warrant the correctness of the material on this page or the sites linked - please take online information and consult with your own medical team to make informed decisions.

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Updated March 29, 2008