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Hematology, Transfusion and Cell Therapy
Article . 2024 . Peer-reviewed
License: CC BY NC ND
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MEDIASTINAL GRAY ZONE LYMPHOMA; SHADES OF GRAY

Authors: Meral Ulukoylu Menguc; Fatma Arıkan; Tayfur Toptas;

MEDIASTINAL GRAY ZONE LYMPHOMA; SHADES OF GRAY

Abstract

Objective: Mediastinal gray zone lymphoma (MGZL) is a rare B cell lymphoma originated from the thymic niche. An incostistency between morphological and immunohistochemical findings is the hallmark of the disease . Both 2022 WHO classification and International Consensus Classification renamed the disease as Mediastinal Gray Zone Lymphoma which excluded non-mediastinal forms. Due to rarity and clinical presentation of mediastinal bulky disease prospective trials for the management of MGZL is limited.Case report: Twenty-nine years old female patient admitted to hospital with dyspnea and night sweats. Basal scans showed an anterior mediastinal mass lesion of 5 × 5 × 6 cm diameter. Tru-cut biopsy of the lesion showed MGZL, cHL -like subtype with immunohistochemically CD 30, CD15, PAX-5 positivity and strong CD20 positive giant cell containing atypical lymphoproliferative mass in a sclerotic background . Background consisted of numerous mature lymphocytes, rare eosinophils, histiocytes and plasma ce Methodology: PET-CT showed anterior mediastinal mass of 8,7 X 6,2 cm standing just behind pericardium with a SUVmax of 28,3 . Along with mediastinal mass right prevascular,preparacardiac and anterior diaphragmatic lympadenopaties of maximum length of 2,5 cm and with a SUVmax ranging between 7,04 and 24,7 were detected. Basal tests showed iron deficiency anemia of hemoglobin 9,8 g/dl and erythrocyte sedimentation rate of 29 mm/hour. LDH was 645 IU/l. Pretherapy echocardiograpy showed pericardial effusion Results: Background consisted of numerous mature lymphocytes, rare eosinophils, histiocytes and plasma cells . PET-CT showed anterior mediastinal mass of 8,7 X 6,2 cm standing just behind pericardium with a SUVmax of 28,3 . Along with mediastinal mass right prevascular,preparacardiac and anterior diaphragmatic lympadenopaties of maximum length of 2,5 cm and with a SUVmax ranging between 7,04 and 24,7 were detected. Basal tests showed iron deficiency anemia of hemoglobin 9,8 g/dl . Conclusion: Targeted therapies especially PD-1 blockage and anti-CD30 therapies are increasingly filling the gap for the management of GZL s as well as cHL and PMBCL. Brentuximab vedotin is a promising agent for the management of GZLs both in the first line and in the relapsed/ refractory setting.

Keywords

Diseases of the blood and blood-forming organs, RC633-647.5

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selected citations
These citations are derived from selected sources.
This is an alternative to the "Influence" indicator, which also reflects the overall/total impact of an article in the research community at large, based on the underlying citation network (diachronically).
BIP!Citations provided by BIP!
popularity
This indicator reflects the "current" impact/attention (the "hype") of an article in the research community at large, based on the underlying citation network.
BIP!Popularity provided by BIP!
influence
This indicator reflects the overall/total impact of an article in the research community at large, based on the underlying citation network (diachronically).
BIP!Influence provided by BIP!
impulse
This indicator reflects the initial momentum of an article directly after its publication, based on the underlying citation network.
BIP!Impulse provided by BIP!
1
Average
Average
Average
gold