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罕见的原发性皮肤滤泡中心淋巴瘤病例,表现为头皮巨大肿瘤,并伴有 JAK2V617F 阳性原发性血小板增多症。

A rare case of primary cutaneous follicle centre lymphoma presenting as a giant tumour of the scalp and combined with JAK2V617F positive essential thrombocythaemia.

机构信息

Department of Haematology, University Hospital of Geneva, Rue Gabrielle-Perret-Gentil 4, 1211 Geneva, Switzerland.

出版信息

Biomark Res. 2014 Apr 1;2(1):7. doi: 10.1186/2050-7771-2-7.

Abstract

Primary cutaneous follicle centre lymphoma (PCFCL) is a rare cutaneous B cell lymphoma in middle-age adults with excellent prognosis. Here we present a case of a patient with a PCFCL in the form of a giant tumour of the scalp in combination with a myeloproliferative neoplasm, JAK2V617F positive essential thrombocythaemia. This case may be of interest because of the favourable outcome in spite of the large size of the PCFCL, the rare combination with essential thrombocythaemia and because it contributes to discussion on the role of JAK2 mutation in such patients.

摘要

原发性皮肤滤泡中心淋巴瘤(PCFCL)是一种罕见的中老年人皮肤 B 细胞淋巴瘤,预后良好。在此,我们报告了一例 PCFCL 患者,其头皮巨大肿瘤合并骨髓增殖性肿瘤、JAK2V617F 阳性原发性血小板增多症。本病例可能具有临床意义,因为尽管 PCFCL 体积较大、罕见合并原发性血小板增多症,但患者预后良好,并且有助于讨论 JAK2 突变在这类患者中的作用。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/8d24/3978001/18cc711b061e/2050-7771-2-7-1.jpg

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