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一名难治性乳糜泻患者的CD103+γδT细胞大颗粒淋巴细胞增多症:一个诊断难题。

CD103+ γδ T cell large granular lymphocytosis in a patient with refractory celiac disease: a diagnostic enigma.

作者信息

Sreedharanunni Sreejesh, Varma Neelam, Sachdeva Man Updesh Singh, Gupta Kirti, Pai Rekha, Kochhar Rakesh, Malhotra Pankaj, Varma Subhash

机构信息

Department of Hematology, Post Graduate Institute of Medical Education and Research (PGIMER), V Floor, SS Anand Block (Research Block A), Chandigarh, 160012, India,

出版信息

Int J Hematol. 2015 Jun;101(6):603-7. doi: 10.1007/s12185-015-1736-x. Epub 2015 Jan 31.

Abstract

Indolent γδ T cell lymphomas/leukemias are rare and overlap with the morphological spectrum of large granular lymphocyte (LGL) leukemia. We report an extremely rare case of CD103(+) γδ T LGL leukemia in a patient with celiac disease who presented with refractory diarrhea. Whether the refractory diarrhea in our patient was a manifestation of LGL leukemia itself or whether the clonal LGL expansion is a manifestation of refractory celiac disease (RCD) remains an enigma. This report highlights the diagnostic difficulties and the need of consensus in categorizing clonal CD103(+) lymphocytosis in patients with RCD.

摘要

惰性γδ T细胞淋巴瘤/白血病较为罕见,在形态学上与大颗粒淋巴细胞(LGL)白血病有重叠。我们报告了1例极为罕见的CD103(+)γδ T LGL白血病病例,患者为乳糜泻,伴有难治性腹泻。我们患者的难治性腹泻是LGL白血病本身的表现,还是克隆性LGL扩增是难治性乳糜泻(RCD)的表现,仍是个谜。本报告强调了在对RCD患者的克隆性CD103(+)淋巴细胞增多进行分类时的诊断困难及达成共识的必要性。

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