Brogan Beth L, Zic John A, Kinney Marsha C, Hu Judy Y, Hamilton Katherine S, Greer John P
Departments of Medicine, Vanderbilt University Medical School, Nashville, Tennessee 37232-5227, USA.
J Am Acad Dermatol. 2003 Aug;49(2):223-8. doi: 10.1067/s0190-9622(03)00889-2.
Large B-cell lymphoma (LBCL) of the leg is an uncommon subset of primary cutaneous B-cell lymphoma that has been described in a series of European patients.
Our purpose was to evaluate the clinical manifestation, diagnostic histopathology, immunophenotype, clinical course, and response to treatment of LBCL of the leg.
We conducted a retrospective case series of 3 patients with primary LBCL of the leg.
The 3 elderly patients presented with progressive erythematous nodules on bilateral or unilateral lower extremities. All 3 patients had pre-existing peripheral edema or peripheral vascular disease. Histopathologic examination of the nodules showed dense lymphocytic infiltrates composed predominantly of large dysplastic lymphocytes that marked as B cells (CD20(+)). In 2 cases, the neoplastic cells were BCL-2 positive. All patients responded to initial therapy with localized electron beam radiation and chemotherapy but had disease progression. One patient had a complete and durable second response to anti-CD20 monoclonal antibody (rituximab).
The patients described have similar clinical and histopathologic features to those previously described. There may be an association between LBCL and pre-existing lower-extremity vascular disease. Treatment of LBCL is difficult, but 1 patient responded well to systemic anti-CD20 monoclonal antibody.
腿部大B细胞淋巴瘤(LBCL)是原发性皮肤B细胞淋巴瘤中一种罕见的亚型,此前在一系列欧洲患者中有所描述。
我们旨在评估腿部LBCL的临床表现、诊断性组织病理学、免疫表型、临床病程及对治疗的反应。
我们对3例原发性腿部LBCL患者进行了回顾性病例系列研究。
3例老年患者双侧或单侧下肢出现进行性红斑结节。所有3例患者均有既往周围性水肿或周围血管疾病。结节的组织病理学检查显示密集的淋巴细胞浸润,主要由标记为B细胞(CD20(+))的大的发育异常淋巴细胞组成。2例患者肿瘤细胞BCL-2阳性。所有患者对局部电子束放疗和化疗的初始治疗均有反应,但疾病仍进展。1例患者对抗CD20单克隆抗体(利妥昔单抗)有完全且持久的二次反应。
所描述的患者具有与先前描述的患者相似的临床和组织病理学特征。LBCL与既往存在的下肢血管疾病之间可能存在关联。LBCL的治疗困难,但1例患者对全身性抗CD20单克隆抗体反应良好。