UMMS Affiliation
Department of Pathology
Publication Date
2017-12-12
Document Type
Article
Disciplines
Diagnosis | Hematology | Hemic and Lymphatic Diseases | Neoplasms | Oncology | Pathological Conditions, Signs and Symptoms | Pathology
Abstract
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.
Rights and Permissions
© 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.
DOI of Published Version
10.1182/bloodadvances.2017009472
Source
Blood Adv. 2017 Dec 11;1(26):2600-2609. doi: 10.1182/bloodadvances.2017009472. eCollection 2017 Dec 12. Link to article on publisher's site
Journal/Book/Conference Title
Blood advances
Related Resources
PubMed ID
29296913
Repository Citation
Pilichowska, Monika; Woda, Bruce A.; Jaffe, Elaine S.; and Evens, Andrew M., "Clinicopathologic consensus study of gray zone lymphoma with features intermediate between DLBCL and classical HL" (2017). Open Access Articles. 3322.
https://escholarship.umassmed.edu/oapubs/3322
Included in
Diagnosis Commons, Hematology Commons, Hemic and Lymphatic Diseases Commons, Neoplasms Commons, Oncology Commons, Pathological Conditions, Signs and Symptoms Commons, Pathology Commons
Comments
Full author list omitted for brevity. For the full list of authors, see article.