Edmonds Nicole, Guerra Ricardo, Noland Mary-Margaret B, Schenck Olivia, Krasner Brett, Gru Alejandro A
Department of Dermatology, University of Virginia, Charlottesville, Virginia, USA.
Departments of Pathology and Dermatology, University of Virginia, Charlottesville, Virginia, USA.
J Cutan Pathol. 2021 Oct;48(10):1311-1316. doi: 10.1111/cup.14079. Epub 2021 Jun 27.
T-cell prolymphocytic leukemia (T-PLL) is an aggressive post-thymic T-cell malignancy, which accounts for 2% of mature lymphocytic leukemias in adults. Though typically presenting with a brief history of B symptoms, hepatosplenomegaly, and marked lymphocytosis, erythematous or nodular skin rashes involving the trunk or limbs may be seen in 25% to 30% of patients, as well as a purpuric rash in a periorbital distribution. Cutaneous involvement typically presents in the context of patients with an established history of T-PLL, but it can less frequently present as an initial symptom heralding the diagnosis. An unusual case of T-PLL is described, presenting initially as palmoplantar ulcerated nodules with an initial biopsy suggestive of perniosis, followed by rapid progression of dark violaceous and bright red papules throughout the body after initiation of obinutuzumab. The diagnosis of T-PLL was subsequently fully supported by the clinical, laboratory, cytologic, and immunophenotypic findings. This case highlights the importance of a multidisciplinary team approach to address such rare and atypical presentations.
T细胞幼淋巴细胞白血病（T-PLL）是一种侵袭性的胸腺后T细胞恶性肿瘤，占成人成熟淋巴细胞白血病的2%。虽然通常表现为有B症状、肝脾肿大和明显淋巴细胞增多的短暂病史，但25%至30%的患者可见累及躯干或四肢的红斑或结节性皮疹，以及眶周分布的紫癜样皮疹。皮肤受累通常出现在已有T-PLL病史的患者中，但较少作为预示诊断的初始症状出现。本文描述了一例不寻常的T-PLL病例，最初表现为掌跖溃疡结节，初始活检提示冻疮，在使用奥滨尤妥珠单抗后，全身迅速出现深紫蓝色和鲜红色丘疹。随后，临床、实验室、细胞学和免疫表型检查结果充分支持了T-PLL的诊断。该病例强调了多学科团队方法在处理此类罕见和非典型表现中的重要性。
