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
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