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 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).
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Resources
The Hematopathology of Lymphoma - technical but full of very good information.
NCI Non-Hodgkin's Lymphoma Information
The Atlas of Genetics and Cytogenetics in Oncology and Haematology page on ALCL
Frontiers in Bioscience is a source of B-cell information.
A May 2003 paper on Anaplastic Lymphoma Kinase (ALK)
Good paper on Cutaneous T-Cell Lymphoma from eMedicine
Information from Hematopathology
Generic Methotrexate information from RxList
Ki-1 Large Cell Lymphoma: A
Clinical Study of 12 Cases , Ezzat et al., January 1995
Lymphomation page on Childhood Lymphomas and page
on T-Cell Lymphomas
A list of research papers by CancerWeb (UK)
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
Related Articles
For more information on Adult Non-Hodgkin's Lymphoma 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
More Useful Information:
Childhood Non-Hodgkin's Lymphoma Information Pages
The Lymphoma Information Network
Books
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
