Cabanillas F, Armitage J, Pugh W C, Weisenburger D, Duvic M
Department of Hematology, University of Texas M.D. Anderson Cancer Center, Houston 77030.
Ann Intern Med. 1995 Feb 1;122(3):210-7. doi: 10.7326/0003-4819-122-3-199502010-00009.
To describe the diagnostic difficulties, response to therapy, and clinical features of lymphomatoid papulosis and the cumulative frequency of transformation to lymphoma.
Case series.
University hospitals.
The records of 21 patients with lymphomatoid papulosis who were seen from 1986 to 1993 were retrieved from the archives of two institutions. The entry criteria for the study were lymphomatoid papulosis misdiagnosed at the time of original presentation or lymphomatoid papulosis that later developed into lymphoma.
When lymphomatoid papulosis tissues are pathologically examined, they are frequently confused with lymphoma, melanoma, or carcinoma. Eight of the 19 patients whose condition was misdiagnosed as malignant received either chemotherapy or radiotherapy. Although lymphomatoid papulosis responded to cytotoxic chemotherapy, the remissions were transient and promptly recurred after or during treatment. However, all five cases that became malignant responded to chemotherapy and have not recurred. Five of 21 patients (24%) developed lymphoma, but the cumulative risk for transformation after 15 years was 80%.
Lymphomatoid papulosis can only be diagnosed accurately through a careful history in which the characteristic waxing and waning of the skin lesions is identified and through proper communication between clinicians and pathologists. Patients with lymphomatoid papulosis have an increased risk for developing lymphoma that is much higher than the 15% to 20% quoted in the literature. Patients who develop lymphoma respond well to cytotoxic chemotherapy and can be cured with appropriate therapy. Internists and oncologists need to be aware of lymphomatoid papulosis and its characteristic clinical features so that this disorder is accurately diagnosed and so that unnecessary and potentially hazardous treatment is avoided.
描述淋巴瘤样丘疹病的诊断难点、对治疗的反应、临床特征以及转化为淋巴瘤的累积发生率。
病例系列研究。
大学医院。
从两家机构的档案中检索出1986年至1993年间诊治的21例淋巴瘤样丘疹病患者的病历。本研究的纳入标准为初诊时误诊为淋巴瘤样丘疹病或后来发展为淋巴瘤的淋巴瘤样丘疹病。
淋巴瘤样丘疹病组织进行病理检查时，常与淋巴瘤、黑色素瘤或癌相混淆。19例被误诊为恶性疾病的患者中，有8例接受了化疗或放疗。虽然淋巴瘤样丘疹病对细胞毒性化疗有反应，但缓解是短暂的，在治疗后或治疗期间很快复发。然而，所有5例恶变的病例对化疗有反应且未复发。21例患者中有5例（24%）发展为淋巴瘤，但15年后转化的累积风险为80%。
淋巴瘤样丘疹病只有通过详细询问病史以识别皮肤损害特征性的消长情况，并通过临床医生与病理医生之间的恰当沟通才能准确诊断。淋巴瘤样丘疹病患者发生淋巴瘤的风险增加，远高于文献报道的15%至20%。发生淋巴瘤的患者对细胞毒性化疗反应良好，适当治疗可治愈。内科医生和肿瘤学家需要了解淋巴瘤样丘疹病及其特征性临床特征，以便准确诊断该疾病并避免不必要的、有潜在风险的治疗。
