Olerud J E, Kulin P A, Chew D E, Carlsen R A, Hammar S P, Weir T W, Patterson S D, Bolen J W, Kadin M E, Barker E
Department of Medicine (Dermatology), University of Washington, Seattle 98195.
Arch Dermatol. 1992 Apr;128(4):501-7. doi: 10.1001/archderm.128.4.501.
Cutaneous T-cell lymphoma (CTCL) frequently presents a difficult diagnostic challenge for the clinician and pathologist. To assess the diagnostic validity of conventional histopathologic findings in CTCL, pretreatment skin biopsy specimens were scored prospectively and independently by a panel of five to seven dermatopathologists and pathologists. Scores were compared with disease outcome. Repeatability of these scores was examined among observers and for the same observer. The study population consisted of 165 subjects, initially referred for suspected mycosis fungoides or Sézary syndrome. Ninety-two patients determined to have CTCL have been followed up for 6.3 +/- 3.5 years (mean +/- SD) and are categorized according to disease outcome: 22 are in complete remission, 35 are in partial remission, three have progressive lymphoma, 15 died of disease, 13 died of other causes, and four were unavailable for follow-up. Seventy-three patients determined not to have CTCL have been followed up for 5.3 +/- 3.2 years without subsequent clinicopathologic evidence of CTCL. These longitudinal data allowed comparisons of the clinical course with the original histologic interpretations.
Data showed that the histologic scores rendered by the pathology panel did not correlate with stage of disease and were not an accurate predictor of clinical outcome, because the histologic ratings did not discriminate between patients who eventually had complete remission and those with either progressive lymphoma or who have died of disease. The results also substantiate the low inherent reliability of histopathologic findings in CTCL. Large differences existed among pathologists in scoring the study populations and repeated reading of selected cases by the same panel member resulted in a change of diagnosis 15% of the time. Among the histologic features evaluated, only the presence of mitoses in the infiltrating cells showed a trend toward an unfavorable outcome.
Pathologic diagnosis in the CTCL disease spectrum should be interpreted with caution and then only in conjunction with the clinical evaluation. As expected, the use of an average value from a panel of readers added a component of stability to the histologic interpretation.
皮肤T细胞淋巴瘤（CTCL）常常给临床医生和病理学家带来诊断难题。为评估CTCL中传统组织病理学发现的诊断有效性，由五至七名皮肤病理学家和病理学家组成的小组对预处理皮肤活检标本进行前瞻性独立评分。将评分与疾病转归进行比较。在观察者之间以及同一观察者对这些评分的可重复性进行了检查。研究人群包括165名最初因疑似蕈样肉芽肿或塞扎里综合征而转诊的受试者。已确定患有CTCL的92例患者已随访6.3±3.5年（平均±标准差），并根据疾病转归进行分类：22例完全缓解，35例部分缓解，3例淋巴瘤进展，15例死于疾病，13例死于其他原因，4例无法进行随访。已确定未患有CTCL的73例患者已随访5.3±3.2年，随后未出现CTCL的临床病理证据。这些纵向数据使得能够将临床病程与最初的组织学解释进行比较。
数据显示，病理小组给出的组织学评分与疾病分期无关，也不是临床转归的准确预测指标，因为组织学分级无法区分最终完全缓解的患者与淋巴瘤进展或死于疾病的患者。结果还证实了CTCL中组织病理学发现的固有可靠性较低。病理学家在对研究人群进行评分时存在很大差异，同一小组成员对选定病例的重复阅读导致15%的诊断发生了改变。在所评估的组织学特征中，只有浸润细胞中存在有丝分裂显示出预后不良的趋势。
对于CTCL疾病谱中的病理诊断应谨慎解读，且仅应结合临床评估进行解读。不出所料，采用一组阅片者的平均值为组织学解释增添了一定的稳定性。
