Grey zone lymphoma - diagnostic and therapeutic challenge

Author:

Agic Danijela1ORCID,Lakic Tanja2ORCID,Nikin Zoran3ORCID

Affiliation:

1. University of Novi Sad, Faculty of Medicine Novi Sad + University Clinical Center of Vojvodina, Clinic for hematology, Novi Sad

2. University of Novi Sad, Faculty of Medicine Novi Sad + Department of Pathology, Novi Sad + University Clinical Center of Vojvodina, Center for pathology and histology, Novi Sad

3. Oncology Institute of Vojvodina, Sremska Kamenica

Abstract

Introduction. ?Grey zone Lymphoma?? is associated with various entities. The last published classification of lymphoproliferative neoplasms includes mediastinal grey zone lymphoma. Precise diagnostic criteria are insufficient and establishing a diagnosis is as complex as deciding on treatment options. In this article pathologist and hematologist discuss issues on this topic through case presentation and literature review. Clinical characteristics. It presents in the younger population usually with a mediastinal mass, sometimes large, with compressive symptoms. Pathologic characteristics. Some cases can?t be classified neither as Hodgkin nor Primary mediastinal B-cell lymphoma. Morphology resembles Hodgkin, but with a positive immunophenotype for primary mediastinal or diffuse large B-cell lymphoma, and vice versa. Case report. We presented a case of a 33-year-old male with cervical lymphadenomegaly, B symptoms and clinical deterioration during the diagnostic period. After the first biopsy, differential diagnosis was Epstein-Barr virus-associated lymphoproliferative disorder or classical Hodgkin lymphoma. The second biopsy confirms Epstein-Barr virus-positive diffuse large B-cell lymphoma. The World Health Organization Classification of lymphoproliferative neoplasms - clinical perspective. The term ?grey zone lymphoma? is associated with overlapping diagnosis or uncertainty in diagnosis in more clinical settings than the ones provided in the 5th World Health Organization Classification. Discussion. For now, chemotherapeutic regimen (rituximab-cyclophos phamide, doxorubicin, vincristine, prednisone) stays the standard first line therapy for diffuse large B-cell lymphoma regardless of the Epstein- Barr virus status. Mediastinal grey zone lymphoma treatment varies: chemotherapeutic regimen (rituximab-cyclophos phamide, doxorubicin, vincristine, prednisone) was linked with better outcomes than chemotherapeutic regimen (adriablastin, bleomycin, vinblastine, dacarbazine) +/-R, but for some patients chemotherapeutic regimen (dose adjusted-etoposide, prednisone, vincristine, cyclophosphamide, doxorubicin) might be beneficial. Conclusion. Grey zone lymphoma is a rare hematologic malignancy that needs extensive sampling for correct diagnosis and is still subject to inter-observer variability.

Publisher

National Library of Serbia

Subject

General Medicine

Reference33 articles.

1. Swerdlow SH, Campo E, Harris NL, Jaffe ES, Pileri SA, Stein H, et al. WHO classification of tumours of haematopoietic and lymphoid tissues. Revised 4th ed. Lyon: IARC; 2017.

2. Alaggio R, Amador C, Anagnostopoulos I, Attygalle AD, Araujo IBO, Berti E, et al. The 5th edition of the World Health Organization classification of haematolymphoid tumours: lymphoid neoplasms. Leukemia. 2022;36(7):1720-48.

3. National Cancer Institute, the surveillance, epidemiology, and end results program. B-cell lymphoma, unclassifiable, with features intermediate between diffuse large B-cell lymphoma and classic Hodgkin lymphoma [Internet]. 2022 [cited 2022 Aug 7]. Available from: https://seer.cancer.gov/seertools/hemelymp h/51f6cf57e3e27c3994bd5333/

4. Rudiger T, Jaffe ES, Delsol G, deWolf-Peeters C, Gascoyne RD, Georgii A, et al. Workshop report on Hodgkin’s disease and related diseases (‘grey zone’ lymphoma). Ann Oncol. 1998;9(Suppl 5):S31-8.

5. Evens AM, Kanakry JA, Sehn LH, Kritharis A, Feldman T, Kroll A, et al. Gray zone lymphoma with features intermediate between classical Hodgkin lymphoma and diffuse large B-cell lymphoma: characteristics, outcomes, and prognostication among a large multicenter cohort. Am J Hematol. 2015;90(9):778-83.

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