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A rare case and diagnostic process of “gray zone” lymphoma

Yanitsa Dimitrova, Kristina Dimitrova, Stefan Radev, Vladimir Dobrev, Vladimir Gerov


Introduction: Gray Zone Lymphoma (GZL) is a rare subtype of lymphoid neoplasm with aggressive development and difficult diagnostics. The term “gray zone lymphoma” has been used to denote a new pathomorphological category of unclassified B – cell lymphoma with intermediate features between diffuse large B-cell lymphoma (DLBCL) and classical Hodgkin’s lymphoma (CHL). It typically occurs in young male and presents as a solitary mediastinal mass, although often its clinical manifestation is not specific. GZL requires a specific treatment approach, because of the fundamental differences in the development of CHL and traditionally more aggressive B – cell lymphoma.

Materials and methods: A clinical case of 50-years-old female, complaining of generalized pruritus is presented. Conducted tests reveal anaemia, increased cholesterol, liver enzymes at the upper limit of the reference values and slightly elevated. A few months later the patient is admitted to a rheumatology clinic with constitutional symptoms and a small–joint inflammation, where conducted computed tomography of the chest revealed a solitary mediastinal mass, which also affects the right lung.

Results: Diagnostic searching necessitates performing of fibrobronchoscopy with lavage and fine needle aspiration, followed by core biopsy. Microbiological testing proved no specific inflammation and cytological examination reveals evidence of Rosai – Dorfman disease. Histological conclusion from the biopsy describes inflammatory myofibroblastic tumor. After upper right lobectomy, pathomorphological examination of the resected material shows classical Hodgkin’s lymphoma of nodular sclerosis subtype (CHL-NS) with primary lung affection, although immunohistochemical analysis and flow cytometry prove expression of tumor markers, normally produced by DLBCL.

Conclusion: These results lead to a new pathological revision, which determined the final diagnosis – Gray Zone Lymphoma. Currently the patient is undergoing treatment with 8 cycles of standard chemotherapy for diffuse large B-cell lymphoma.


gray zone lymphoma, diffuse large B-cell lymphoma, Hodgkin’s lymphoma, diagnostic, treatment approach, R-CHOP



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