Ebo D, Van Hoof A
Acta Clin Belg. 1992;47(3):170-7. doi: 10.1080/17843286.1992.11718227.
The "large cell anaplastic lymphoma, Ki-1 positive" is a recently described lymphoma subtype (about 1-8% of all NHL). Distinction from Hodgkin's disease and true histiocytic lymphoma/malignant histiocytosis is not always possible, even by experienced pathologists. It was recently incorporated in the updated Kiel Classification of lymphomas. Classical histologic appearance is a sinusoidal growth pattern in lymph nodes and presence of large bizarre anaplastic cells. Use of cell markers LCA, EMA and Ki-1 or Ber-H2 is essential for diagnosis. The mean age of patients is 50 years. Approximately 50% of patients have an advanced stage (III-IV). Prognosis depends on age and tumor localisations. Cutaneous involvement only is usually associated with a good prognosis. Median survival for patients with extra-cutaneous disease is 13 months. Treatment with intensive chemotherapy is usually needed. Long term remissions are more frequently seen in children and adolescents.
“大细胞间变性淋巴瘤，Ki-1阳性”是一种最近才被描述的淋巴瘤亚型（约占所有非霍奇金淋巴瘤的1-8%）。即使是经验丰富的病理学家，也并非总能将其与霍奇金病及真性组织细胞淋巴瘤/恶性组织细胞增生症区分开来。它最近被纳入了更新后的淋巴瘤 Kiel 分类法中。典型的组织学表现为淋巴结内的窦状生长模式以及存在大的怪异间变性细胞。使用细胞标志物LCA、EMA和Ki-1或Ber-H2对于诊断至关重要。患者的平均年龄为50岁。约50%的患者处于晚期（III-IV期）。预后取决于年龄和肿瘤定位。仅皮肤受累通常预后良好。皮肤外疾病患者的中位生存期为13个月。通常需要强化化疗进行治疗。儿童和青少年更常出现长期缓解。
