Disease Profile

Anaplastic large cell lymphoma

Prevalence ?
Prevalence estimates on Rare Medical Network websites are calculated based on data available from numerous sources, including US and European government statistics, the NIH, Orphanet, and published epidemiologic studies. Rare disease population data is recognized to be highly variable, and based on a wide variety of source data and methodologies, so the prevalence data on this site should be assumed to be estimated and cannot be considered to be absolutely correct.
1-9 / 100 000

3,310 - 29,790

US Estimated

1-9 / 100 000

46,215 - 5,135

Europe Estimated

Age of Onset

All ages

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ICD-10

C84.6 C84.7

Inheritance

Autosomal dominant ?A pathogenic variant in only one gene copy in each cell is sufficient to cause an autosomal dominant disease

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Autosomal recessive ?Pathogenic variants in both copies of each gene of the chromosome are needed to cause an autosomal recessive disease and observe the mutant phenotype

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X-linked dominant ?X-linked dominant inheritance, sometimes referred to as X-linked dominance, is a mode of genetic inheritance by which a dominant gene is carried on the X chromosome.

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X-linked recessive ?Pathogenic variants in both copies of a gene on the X chromosome cause an X-linked recessive disorder

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Mitochondrial or multigenic ?Mitochondrial genetic disorders can be caused by changes (mutations) in either the mitochondrial DNA or nuclear DNA that lead to dysfunction of the mitochondria and inadequate production of energy.

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Multigenic or multifactor ?Inheritance involving many factors, of which at least one is genetic but none is of overwhelming importance, as in the causation of a disease by multiple genetic and environmental factors.

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Not applicable

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Other Names (AKA)

ALCL

Categories

Rare Cancers

Summary

Anaplastic large cell lymphoma (ALCL) is a rare type of Non-Hodgkins lymphoma. Lymphoma is a cancer of the lymph system, part of our immune system. Non-Hodgkins lymphoma involves abnormal growth of white blood cells, either T cells or B cells. Anaplastic large cell lymphoma is an aggressive cancer that usually involves the T-cells. Cancer cells in ALCL can be identified by their appearance under the microscope and by the presence of a tumor marker called CD30 or Ki-1.[1][2]

There are two types of ALCL, a type that affects mainly the skin (cutaneous ALCL) and a type that affects other body organs (systmic ALCL). Systemic ALCL also has two types, ALK-positive (anaplastic lymphoma kinase) and ALK-negative. ALK-positive ALCL occurs more often in children and young adults. ALK-negative ALCL tends to occur in older adults.[2][3]

The symptoms of cutaneous ALCL include red skin lesions that break open and do not heal. Sometimes cutaneous ALCL is also found in the lymph nodes. The symptoms of systemic ALCL include fever, night sweats, and weight loss (B symptoms). The cancer can be found in the bone, soft tissue, spleen, liver and skin. The cause of ALCL is unknown. One type of ALK-negative ALCL has been associated with breast implants. ALCL is diagnosed by a biopsy of the tumor or abnormal skin and examination of the tumor cells under a microscope. Additional testing including PET scans, CT scans, MRI and a bone marrow biopsy can tell doctors if the cancer has spread to other organs.[15060[15061] 

ALCL is treated using chemotherapy, and a stem-cell transplant for people with more aggressive cancer or for ALCL that has come back. In addition, there are newer treatments that target the cancer cells directly and have been very effective. In general, ALK-positive ALCL has a better long-term outlook than ALK-negative ALCL.[15061][15062]

Treatment

FDA-Approved Treatments

The medication(s) listed below have been approved by the Food and Drug Administration (FDA) as orphan products for treatment of this condition. Learn more orphan products.

  • Brentuximub vedotin(Brand name: Adcetris) Manufactured by Seattle Genetics, Inc
    FDA-approved indication: November 2018, brentuximab vedotin (Adcetris) was approved for the treatment of adult patients with previously untreated systemic anaplastic large cell lymphoma (sALCL) or other CD30-expressing peripheral T-cell lymphomas (PTCL), including angioimmunoblastic T-cell lymphoma and PTCL not otherwise specified, in combination with cyclophosphamide, doxorubicin, and prednisone. In November 2016, it was approved for treatment of adult patients with primary cutaneous anaplastic large cell lymphoma. In August 2011, it was approved for the treatment of patients with systemic anaplastic large cell lymphoma (sALCL) after failure of at least one prior multi-agent chemotherapy regimen.
    National Library of Medicine Drug Information Portal
    Medline Plus Health Information

Organizations

Support and advocacy groups can help you connect with other patients and families, and they can provide valuable services. Many develop patient-centered information and are the driving force behind research for better treatments and possible cures. They can direct you to research, resources, and services. Many organizations also have experts who serve as medical advisors or provide lists of doctors/clinics. Visit the group’s website or contact them to learn about the services they offer. Inclusion on this list is not an endorsement by GARD.

Organizations Supporting this Disease

    Learn More

    These resources provide more information about this condition or associated symptoms. The in-depth resources contain medical and scientific language that may be hard to understand. You may want to review these resources with a medical professional.

    In-Depth Information

    • The Monarch Initiative brings together data about this condition from humans and other species to help physicians and biomedical researchers. Monarch’s tools are designed to make it easier to compare the signs and symptoms (phenotypes) of different diseases and discover common features. This initiative is a collaboration between several academic institutions across the world and is funded by the National Institutes of Health. Visit the website to explore the biology of this condition.
    • Online Mendelian Inheritance in Man (OMIM) is a catalog of human genes and genetic disorders. Each entry has a summary of related medical articles. It is meant for health care professionals and researchers. OMIM is maintained by Johns Hopkins University School of Medicine. 
    • PubMed is a searchable database of medical literature and lists journal articles that discuss Anaplastic large cell lymphoma. Click on the link to view a sample search on this topic.

      References

      1. Shustov A, Soma L. Anaplastic large cell lymphoma: Contemporary concepts and optimal management.. Cancer Treat Res. 2019; 176:127-144. https://www.ncbi.nlm.nih.gov/pubmed/30596216.
      2. Tsuyama N, Sakamoto K, Sakata S, Dobashi A, Takeuchi K. Anaplastic large cell lymphoma: pathology, genetics and clinical aspects.. J Clin Exp Hematop. 2017; 57(3):120-142. https://www.ncbi.nlm.nih.gov/pubmed/29279550.
      3. Ferreri AJ, Govi S, Pileri SA, Savage KJ. Anaplastic large cell lymphoma, ALK-negative. Crit Rev Oncol Hematol. 2013; 85(2):206-215. https://www.ncbi.nlm.nih.gov/pubmed/22789917.