{"title":"MEDIASTINAL GRAY ZONE LYMPHOMA; SHADES OF GRAY","authors":"Meral Ulukoylu Menguc , Fatma Arıkan , Tayfur Toptas","doi":"10.1016/j.htct.2024.04.031","DOIUrl":null,"url":null,"abstract":"<div><h3>Objective</h3><p>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.</p><p>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</p></div><div><h3>Methodology</h3><p>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</p></div><div><h3>Results</h3><p>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 .</p></div><div><h3>Conclusion</h3><p>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.</p></div>","PeriodicalId":12958,"journal":{"name":"Hematology, Transfusion and Cell Therapy","volume":null,"pages":null},"PeriodicalIF":1.8000,"publicationDate":"2024-05-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.sciencedirect.com/science/article/pii/S2531137924001135/pdfft?md5=0de6476f0a2b8aad50379ba7c9d68294&pid=1-s2.0-S2531137924001135-main.pdf","citationCount":"0","resultStr":null,"platform":"Semanticscholar","paperid":null,"PeriodicalName":"Hematology, Transfusion and Cell Therapy","FirstCategoryId":"1085","ListUrlMain":"https://www.sciencedirect.com/science/article/pii/S2531137924001135","RegionNum":0,"RegionCategory":null,"ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":null,"EPubDate":"","PubModel":"","JCR":"Q3","JCRName":"HEMATOLOGY","Score":null,"Total":0}
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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.
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