Iványi J L, Marton E, Szabó F, Mahunka M, Kelényi G
Vas Megyei Markusovszky Kórház, Hematológiai Osztály, Szombathely.
Orv Hetil. 1997 Mar 30;138(13):779-84.
CD30(Ki-1) positive anaplastic large cell lymphoma (ALCL) is a distinct entity, in which the monoclonal antibody-positivity against the CD30(Ki-1) antigen of tumour cells has a diagnostic value. The histological subtypes of ALCL show also certain clinical differences. Except for some pediatric cases and cutaneous forms clinical outcome is very unfavourable despite of the various treatment methods. In this prospective study (follow-up of 11-60, median 16 months) clinicopathological data and treatment results of fifteen adult patients with ALCL were analysed, Mean age was 46 (16-69) ys with a bimodal tendency and a distinct female: male ratio (3:2) was observed. Early clinical stages (I-II/A-B, eight patients) dominated, and two main groups could be distinguished histologically (Hodgkin-related, ALCL-HR and common type, -CT in eight and seven patients), respectively. In all histological specimens CD30 antigen expression was detected. Additional immunophenotyping was performed in five cases (two 0-variant, two of B-cell and one of T-cell origin), respectively. A bulky disease, mainly in the mediastinum was observed in six cases, and a primary gastrointestinal localization in two other patients. In the treatment of these high grade malignant lymphomas a combination of cobalt irradiation and aggressive chemotherapy was applied (in elder the CHOP-regimen, in younger patients mainly the Pro-MACE-Cyta-BOM-protocol). In one relapsed younger patient autologous bone marrow transplantation was also performed. A complete or partial remission was achieved in thirteen patients (86.6%) but six patients expired after only a short response period to therapy. Overall survival was 19, whilst disease-free survival revealed to be 15 months. Eight of their living nine patients have a durable complete remission. Due to residual mediastinal mass after radiotherapy in three cases a permanent radiological follow-up is needed. Advanced age and clinical stages are considered to be unfavourable, whilst histological subtypes were indifferent prognostic factors, as well. Favourable results in therapy and durable complete remission in younger patients are probably caused by the their better tolerance of third-line aggressive chemotherapy.
CD30(Ki-1)阳性间变性大细胞淋巴瘤(ALCL)是一种独特的实体瘤,其中肿瘤细胞对CD30(Ki-1)抗原的单克隆抗体阳性具有诊断价值。ALCL的组织学亚型也显示出一定的临床差异。除了一些儿科病例和皮肤型外,尽管有各种治疗方法,但临床结果仍然非常不理想。在这项前瞻性研究(随访11 - 60个月,中位随访16个月)中,分析了15例成年ALCL患者的临床病理数据和治疗结果。平均年龄为46岁(16 - 69岁),呈双峰趋势,且观察到明显的女性与男性比例(3:2)。早期临床分期(I - II/A - B,8例患者)占主导,组织学上可分为两个主要组(霍奇金相关型,ALCL - HR和常见型,-CT,分别为8例和7例患者)。在所有组织学标本中均检测到CD30抗原表达。另外对5例病例(2例0变异型、2例B细胞起源和1例T细胞起源)进行了免疫表型分析。6例患者观察到有大包块病变,主要位于纵隔,另外2例患者为原发性胃肠道定位。在这些高级别恶性淋巴瘤的治疗中,采用了钴放疗和积极化疗相结合的方法(年龄较大者采用CHOP方案,较年轻患者主要采用Pro - MACE - Cyta - BOM方案)。1例复发的年轻患者还进行了自体骨髓移植。13例患者(86.6%)实现了完全或部分缓解,但6例患者在对治疗仅有短暂反应期后死亡。总生存期为19个月,无病生存期为15个月。9例存活患者中有8例实现了持久的完全缓解。3例患者放疗后因纵隔残留肿块需要进行长期影像学随访。高龄和临床分期被认为是不利因素,而组织学亚型也是预后无关因素。年轻患者治疗效果良好且实现持久完全缓解可能是因为他们对三线积极化疗的耐受性更好。