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dc.contributor.authorPilichowska, Monika
dc.contributor.authorWoda, Bruce A.
dc.contributor.authorJaffe, Elaine S.
dc.contributor.authorEvens, Andrew M.
dc.date2022-08-11T08:09:49.000
dc.date.accessioned2022-08-23T16:44:32Z
dc.date.available2022-08-23T16:44:32Z
dc.date.issued2017-12-12
dc.date.submitted2018-03-29
dc.identifier.citation<p>Blood Adv. 2017 Dec 11;1(26):2600-2609. doi: 10.1182/bloodadvances.2017009472. eCollection 2017 Dec 12. <a href="https://doi.org/10.1182/bloodadvances.2017009472">Link to article on publisher's site</a></p>
dc.identifier.issn2473-9529 (Linking)
dc.identifier.doi10.1182/bloodadvances.2017009472
dc.identifier.pmid29296913
dc.identifier.urihttp://hdl.handle.net/20.500.14038/40520
dc.description<p>Full author list omitted for brevity. For the full list of authors, see article.</p>
dc.description.abstractGray zone lymphoma (GZL) is described as sharing features with classical Hodgkin lymphoma (cHL) and diffuse large B-cell lymphoma (DLBCL). However, there remains complexity in establishing diagnosis, delineating prognosis, and determining optimum therapy. Sixty-eight cases diagnosed as GZL across 15 North American academic centers were evaluated by central pathology review to achieve consensus. Of these, only 26 (38%) were confirmed as GZL. Morphology was critical to GZL consensus diagnosis (eg, tumor cell richness); immunohistochemistry showed universal B-cell derivation, frequent CD30 expression, and rare Epstein-Barr virus (EBV) positivity (CD20(+), 83%; PAX5(+), 100%; BCL6(+), 20%; MUM1(+), 100%; CD30(+), 92%; EBV(+), 4%). Forty-two cases were reclassified: nodular sclerosis (NS) cHL, n = 27 (including n = 10 NS grade 2); lymphocyte predominant HL, n = 4; DLBCL, n = 4; EBV(+) DLBCL, n = 3; primary mediastinal large BCL n = 2; lymphocyte-rich cHL and BCL-not otherwise specified, n = 1 each. GZL consensus-confirmed vs reclassified cases, respectively, more often had mediastinal disease (69% vs 41%; P = .038) and less likely more than 1 extranodal site (0% vs 25%; P = .019). With a 44-month median follow-up, 3-year progression-free survival (PFS) and overall survival for patients with confirmed GZL were 39% and 95%, respectively, vs 58% and 85%, respectively, for reclassified cases (P = .19 and P = .15, respectively). Interestingly, NS grade 2 reclassified patients had similar PFS as GZL consensus-confirmed cases. For prognostication of GZL cases, hypoalbuminemia was a negative factor (3-year PFS, 12% vs 64%; P = .01), whereas frontline cyclophosphamide, doxorubicin, vincristine, and prednisone +/- rituximab (CHOP+/-R) was associated with improved 3-year PFS (70% vs 20%; P = .03); both factors remained significant on multivariate analysis. Altogether, accurate diagnosis of GZL remains challenging, and improved therapeutic strategies are needed.
dc.language.isoen_US
dc.relation<p><a href="http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?db=pubmed&cmd=Retrieve&list_uids=29296913&dopt=Abstract">Link to Article in PubMed</a></p>
dc.rights© Blood Advances Online by the American Society of Hematology. Open access. Publisher PDF posted as allowed by the publisher's author rights policy at http://www.bloodadvances.org/page/authors/copyright-information.
dc.subjectDiagnosis
dc.subjectHematology
dc.subjectHemic and Lymphatic Diseases
dc.subjectNeoplasms
dc.subjectOncology
dc.subjectPathological Conditions, Signs and Symptoms
dc.subjectPathology
dc.titleClinicopathologic consensus study of gray zone lymphoma with features intermediate between DLBCL and classical HL
dc.typeJournal Article
dc.source.journaltitleBlood advances
dc.source.volume1
dc.source.issue26
dc.identifier.legacyfulltexthttps://escholarship.umassmed.edu/cgi/viewcontent.cgi?article=4333&amp;context=oapubs&amp;unstamped=1
dc.identifier.legacycoverpagehttps://escholarship.umassmed.edu/oapubs/3322
dc.identifier.contextkey11871274
refterms.dateFOA2022-08-23T16:44:32Z
html.description.abstract<p>Gray zone lymphoma (GZL) is described as sharing features with classical Hodgkin lymphoma (cHL) and diffuse large B-cell lymphoma (DLBCL). However, there remains complexity in establishing diagnosis, delineating prognosis, and determining optimum therapy. Sixty-eight cases diagnosed as GZL across 15 North American academic centers were evaluated by central pathology review to achieve consensus. Of these, only 26 (38%) were confirmed as GZL. Morphology was critical to GZL consensus diagnosis (eg, tumor cell richness); immunohistochemistry showed universal B-cell derivation, frequent CD30 expression, and rare Epstein-Barr virus (EBV) positivity (CD20(+), 83%; PAX5(+), 100%; BCL6(+), 20%; MUM1(+), 100%; CD30(+), 92%; EBV(+), 4%). Forty-two cases were reclassified: nodular sclerosis (NS) cHL, n = 27 (including n = 10 NS grade 2); lymphocyte predominant HL, n = 4; DLBCL, n = 4; EBV(+) DLBCL, n = 3; primary mediastinal large BCL n = 2; lymphocyte-rich cHL and BCL-not otherwise specified, n = 1 each. GZL consensus-confirmed vs reclassified cases, respectively, more often had mediastinal disease (69% vs 41%; P = .038) and less likely more than 1 extranodal site (0% vs 25%; P = .019). With a 44-month median follow-up, 3-year progression-free survival (PFS) and overall survival for patients with confirmed GZL were 39% and 95%, respectively, vs 58% and 85%, respectively, for reclassified cases (P = .19 and P = .15, respectively). Interestingly, NS grade 2 reclassified patients had similar PFS as GZL consensus-confirmed cases. For prognostication of GZL cases, hypoalbuminemia was a negative factor (3-year PFS, 12% vs 64%; P = .01), whereas frontline cyclophosphamide, doxorubicin, vincristine, and prednisone +/- rituximab (CHOP+/-R) was associated with improved 3-year PFS (70% vs 20%; P = .03); both factors remained significant on multivariate analysis. Altogether, accurate diagnosis of GZL remains challenging, and improved therapeutic strategies are needed.</p>
dc.identifier.submissionpathoapubs/3322
dc.contributor.departmentDepartment of Pathology
dc.source.pages2600-2609


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