Xiao Amy, Beatty Colleen J, Choudhary Sonal, Akilov Oleg E
School of Medicine, University of Pittsburgh, Pittsburgh, PA 15260, USA.
Department of Dermatology, University of Pittsburgh, Pittsburgh, PA 15261-2109, USA.
Hematol Rep. 2023 Sep 1;15(3):513-517. doi: 10.3390/hematolrep15030053.
Primary cutaneous diffuse large B-cell lymphoma, leg type (PCDLBCL-LT), is a rare and aggressive variant of primary cutaneous lymphoma that typically expresses B cells as well as MUM1/IRF4, BCL2, and FOXP1, whereas BCL6 may be present or undetectable. We present a case of CD5+ PCDLBCL-LT presenting as a 6 mm pink-bluish nodule on the mid-left thigh, which was concerning for basal cell carcinoma. The histological examination reveals the presence of an intradermal proliferation of large, atypical CD5+, CD20+ BCL2+, BCL6+, MUM-1+, and Cyclin-D1+ lymphocytes in a nodular, diffuse interstitial and perivascular distribution. Because the patient presented with a small, single nodule, the systemic treatment of multiagent chemotherapy was avoided and localized electron beam radiation therapy with rituximab was initiated instead, achieving complete response. Early identification of PCDLBCL-LT is key for maximal therapeutic benefit and prognosis; it is important to consider PCDLBCL-LT on the differential when evaluating small, single nodules on the lower extremities of elderly patients.
原发性皮肤弥漫性大B细胞淋巴瘤,腿部型(PCDLBCL-LT),是原发性皮肤淋巴瘤的一种罕见且侵袭性变体,通常表达B细胞以及MUM1/IRF4、BCL2和FOXP1,而BCL6可能存在也可能检测不到。我们报告一例CD5+ PCDLBCL-LT病例,表现为左大腿中部一个6毫米的粉蓝色结节,最初怀疑为基底细胞癌。组织学检查显示真皮内有大量非典型CD5+、CD20+、BCL2+、BCL6+、MUM-1+和细胞周期蛋白D1+淋巴细胞呈结节状、弥漫性间质和血管周围分布。由于患者仅表现为一个小的单发性结节,因此避免了多药联合化疗的全身治疗,转而开始使用利妥昔单抗进行局部电子束放射治疗,最终实现了完全缓解。早期识别PCDLBCL-LT是获得最大治疗益处和良好预后的关键;在评估老年患者下肢的小的单发性结节时,将PCDLBCL-LT纳入鉴别诊断很重要。