de Figueiredo Rafael Henriques, Parreira Beatriz Simão, Canão Pedro Amoroso, Cardoso Leila, Fonseca Elsa, Almeida Jorge
Internal Medicine department, Centro Hospitalar e Universitário de São João, Porto, Portugal.
Anatomical Pathology department, Centro Hospitalar e Universitário de São João, Porto, Portugal.
Arch Clin Cases. 2022 Dec 19;9(4):140-144. doi: 10.22551/2022.37.0904.10220. eCollection 2022.
Peripheral T-cell lymphoma, not otherwise specified (PTCL-NOS) is the most common subgroup of peripheral T-cell lymphomas (PTCL), and constitutes a diagnosis of exclusion. At presentation, most patients exhibit B symptoms and generalized lymphadenopathy, with or without concomitant extra-nodal involvement. We present a case of a man admitted to the hospital with B symptoms, generalized lymphadenopathy and a pruritic exanthema. Laboratory workup reveled persistent eosinophilia and malignant hypercalcemia. The excisional lymph node biopsy diagnosed PTCL-NOS, and the skin biopsy demonstrated a lichenoid dermatitis, compatible with the presumptive clinical diagnosis of a drug reaction with eosinophilia and systemic symptoms (DRESS) syndrome. The patient was treated with topical betamethasone with good overall response, and initiated the first cycle of chemotherapy before discharge. This case report describes a PTCL-NOS with a concomitant non-lymphoproliferative disease, the challenging diagnostic workup of the two diseases and reinforces the most important features of the lymphoproliferative neoplasm.
外周T细胞淋巴瘤,非特指型(PTCL-NOS)是外周T细胞淋巴瘤(PTCL)最常见的亚组,属于排除性诊断。就诊时,大多数患者表现为B症状和全身淋巴结肿大,伴或不伴有结外受累。我们报告一例男性患者,因B症状、全身淋巴结肿大和瘙痒性皮疹入院。实验室检查显示持续性嗜酸性粒细胞增多和恶性高钙血症。切除性淋巴结活检诊断为PTCL-NOS,皮肤活检显示苔藓样皮炎,符合伴有嗜酸性粒细胞增多和全身症状的药物反应(DRESS)综合征的临床推测诊断。患者接受外用倍他米松治疗,总体反应良好,并在出院前开始了第一个化疗周期。本病例报告描述了一例伴有非淋巴增殖性疾病的PTCL-NOS、两种疾病具有挑战性的诊断过程,并强化了淋巴增殖性肿瘤的最重要特征。