Rare Pediatrics News

Disease Profile

Gray zone 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.

Unknown

Age of onset

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ICD-10

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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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Categories

Rare Cancers

Summary

Gray zone lymphoma is a rare type of lymphoma, cancer of a part of the immune system called the lymph system. It is called "gray zone" lymphoma because it has features intermediate between classical Hodgkin lymphoma and diffuse large B-cell lymphoma (DLBCL), but cannot be assigned specifically to either type.[1][2] In many cases, the original diagnosis of gray zone lymphoma is later reclassified as a different type of lymphoma, such as nodular sclerosis classical Hodgkin lymphoma (NScHL).[1] An accurate diagnosis of gray zone lymphoma is challenging, and the clinical characteristics, optimum therapy, and prognosis have not been well-defined.[1] While some features suggest it should be treated like Hodgkin lymphoma, other features suggest it should be treated like DLBCL.[3]

Treatment

Gray zone lymphoma shares features with two other types of lymphoma, classical Hodgkin lymphoma (cHL) and diffuse large B-cell lymphoma (DLBCL). Because these lymphomas are treated differently, the optimal therapy for gray zone lymphoma is unclear. Because there are no consensus guidelines for the best treatment of gray zone lymphoma, treatment is currently determined based on each individual's diagnosis.[4] In general, treatment usually involves chemotherapy (with the specific drugs depending upon each person's treatment plan), which may be followed by radiation therapy in some cases.[4] Some research has suggested that treatment with DLBCL-based regimens is the most effective for some people (such as the drug combination R-CHOP or dose-adjusted EPOCH-R), but additional research is needed.[1] Individual case reports have reported success with different PD-1 inhibitors (such as pembrolizumab and nivolumab), but larger studies are needed to determine if this might be an effective new strategy for treating gray zone lymphoma.[5]

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

    Social Networking Websites

      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.

      Where to Start

        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.
        • PubMed is a searchable database of medical literature and lists journal articles that discuss Gray zone lymphoma. Click on the link to view a sample search on this topic.

          References

          1. Pilichowska M, Pittaluga S, Ferry JA, et al. Clinicopathologic consensus study of gray zone lymphoma with features intermediate between DLBCL and classical HL. Blood Adv. December 11, 2017; 1(26):2600-2609. https://www.bloodadvances.org/content/1/26/2600?sso-checked=true.
          2. Kieron Dunleavy and Wyndham H. Wilson. Primary mediastinal B-cell lymphoma and mediastinal gray zone lymphoma: do they require a unique therapeutic approach?. Blood. January, 2015; 125(1):33-39.
          3. Wilson WH, et. al. A prospective study of mediastinal gray-zone lymphoma. Blood. September 4, 2014; 124(10):1563-1569.
          4. Traverse-Glehen A, Pittaluga S, Gaulard P, Sorbara L, Alonso MA, Raffeld M, Jaffe ES. Mediastinal gray zone lymphoma: the missing link between classic Hodgkin's lymphoma and mediastinal large B-cell lymphoma. American Journal of Surgical Pathology. 2005; 29:1411-1421. https://www.ncbi.nlm.nih.gov/pubmed/16224207. Accessed 5/12/2012.
          5. Melani C, Major A, Schowinsky J, et al. PD-1 Blockade in Mediastinal Gray-Zone Lymphoma. N Engl J Med. July 6, 2017; 377(1):89-91. https://www.nejm.org/doi/full/10.1056/NEJMc1704767.

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