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特发性骨髓纤维化患者伴假性Pelger-Huet 异常的坏疽性脓皮病。

Pyoderma gangrenosum associated with pseudo-Pelger-Huet anomaly in a patient with idiopathic myelofibrosis.

机构信息

Department of Medicine, Section of Dermatology, University of Verona, Verona, Italy.

出版信息

Dermatol Online J. 2022 Dec 15;28(6). doi: 10.5070/D328659742.

Abstract

Pseudo-Pelger-Huët anomaly is a condition in which almost all the granulocytes are hyposegmented and/or hypogranulated. It is typically recognized in peripheral blood smears and represents a marker of several disorders, such as myeloproliferative diseases and myelodysplasia. The occurrence of the pseudo-Pelger-Huët anomaly in the cutaneous infiltrate of pyoderma gangrenosum is very rare. We describe the case of a 70-year-old man with idiopathic myelofibrosis who developed pyoderma gangrenosum. Histological examination showed an infiltrate consisting of granulocytic elements with features of dysmaturity and segmentation anomalies (hypo- and hypersegmented forms), suggestive of pseudo-Pelger-Huët anomaly. Methylprednisolone treatment resulted in progressive improvement of pyoderma gangrenosum.

摘要

假性 Pelger-Huët 异常是一种几乎所有粒细胞都呈轻度分叶和/或颗粒减少的情况。它通常在外周血涂片上被识别,代表了几种疾病的标志物,如骨髓增生性疾病和骨髓增生异常。假 Pelger-Huët 异常在坏疽性脓皮病的皮肤浸润中非常罕见。我们描述了一例 70 岁男性,患有特发性骨髓纤维化,发生坏疽性脓皮病。组织学检查显示浸润由粒细胞成分组成,具有不成熟和分叶异常的特征(低和高 分叶形式),提示假性 Pelger-Huët 异常。甲泼尼龙治疗导致坏疽性脓皮病逐渐改善。

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