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一例以发热和结节性红斑样皮损为表现,最终确诊为γ-δ T细胞淋巴瘤并合并噬血细胞性淋巴组织细胞增生症的病例。

A Case of Fever and Erythema Nodosum-Like Lesions Leading to a New Diagnosis of Gamma-Delta T-Cell Lymphoma Complicated by Hemophagocytic Lymphohistiocytosis.

作者信息

Murphy William S, Yeh Jennifer E, Nazarian Rosalynn M, Sohani Aliyah R, Kvedar Joseph, Kroshinsky Daniela

机构信息

Harvard Medical School, Harvard Business School, Boston, Massachusetts, USA.

Harvard Combined Dermatology Residency Program, Boston, Massachusetts, USA.

出版信息

Dermatopathology (Basel). 2020 Feb 18;6(4):266-270. doi: 10.1159/000505471. eCollection 2019 Oct-Dec.

Abstract

A 69-year-old Vietnamese female presented with fever and new-onset tender subcutaneous nodules on her trunk and lower extremities initially thought to be clinically consistent with erythema nodosum. A biopsy showed an atypical, predominantly lobular lymphocytic panniculitis with admixed neutrophils, karyorrhectic debris, and histiocytes with subcutaneous fat necrosis. Immunohistochemistry was consistent with gamma-delta T-cell lymphoma. The patient was initiated on a chemotherapy regimen of cyclophosphamide, doxorubicin, vincristine, etoposide, and prednisone (CHOEP) with partial remission, and is currently undergoing evaluation for bone marrow transplant. This case highlights the ability of cutaneous gamma-delta T-cell lymphoma to mimic more common cutaneous conditions such as erythema nodosum, and stresses the importance of a broad differential for new presentation of tender subcutaneous nodules with concomitant systemic symptoms.

摘要

一名69岁的越南女性,躯干和下肢出现发热及新发的压痛性皮下结节,最初临床认为与结节性红斑相符。活检显示为非典型的、以小叶为主的淋巴细胞性脂膜炎,伴有中性粒细胞、核碎裂碎片及组织细胞混合存在,皮下脂肪坏死。免疫组化结果符合γ-δ T细胞淋巴瘤。患者开始接受环磷酰胺、阿霉素、长春新碱、依托泊苷及泼尼松(CHOEP)化疗方案,部分缓解,目前正在接受骨髓移植评估。该病例凸显了皮肤γ-δ T细胞淋巴瘤模仿更常见皮肤疾病如结节性红斑的能力,并强调了对于伴有全身症状的新发压痛性皮下结节进行广泛鉴别诊断的重要性。

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